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Artykuły w czasopismach na temat „XLQ”

Autor: Grafiati
Data publikacji: 4 czerwca 2021
Data aktualizacji: 9 lutego 2022

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1

Cai, Hongcai, Guowei Zhang, Zechen Yan, and Xuejun Shang. "The Effect of Xialiqi Capsule on Testosterone-Induced Benign Prostatic Hyperplasia in Rats." Evidence-Based Complementary and Alternative Medicine 2018 (September 30, 2018): 1–9. http://dx.doi.org/10.1155/2018/5367814.

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Benign prostatic hyperplasia (BPH) is common among elderly men, of which inflammation, oxidative stress, proliferative, and apoptotic changes play important roles. Xialiqi (XLQ) capsule, a traditional Chinese herbal formula, is used as a potential drug in treating BPH. This study aims to evaluate the therapeutic effect of XLQ capsule on testosterone propionate- (TP-) induced BPH in rats. Fifty male Sprague-Dawley rats were randomly divided into 5 groups: sham control, BPH model, high and low dose of XLQ, and finasteride as a positive control group. All groups were treated with appropriate drug
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2

Yang, Feiya, Lingquan Meng, Panpan Han, Dexi Chen, Mingshuai Wang, Yongguang Jiang, Yanqiao Wu, Yiling Wu, and Nianzeng Xing. "New therapy with XLQ ® to suppress chronic prostatitis through its anti‐inflammatory and antioxidative activities." Journal of Cellular Physiology 234, no. 10 (February 20, 2019): 17570–77. http://dx.doi.org/10.1002/jcp.28380.

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Vallejo, César. "Trilce XLI‐XLV." Review: Literature and Arts of the Americas 25, no. 45 (January 1991): 77–81. http://dx.doi.org/10.1080/08905769108594327.

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Pouzar, Vladimír, Hana Chodounská, Dalibor Sameš, Pavel Drašar та Miroslav Havel. "Derivatives of 5α-androstan-3α- and 3β-ol with acrylate side chain". Collection of Czechoslovak Chemical Communications 55, № 5 (1990): 1243–56. http://dx.doi.org/10.1135/cccc19901243.

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Hydroxy derivatives I, II, III, XVII and XX were oxidized to give the respective aldehydes IV, V, VI, XVIII and XXI which were further converted by Wittig-Horner reaction into unsaturated methyl and ethyl esters. Removal of the acetal protecting group in position 3 afforded methylesters X, XXIV and XXXVI and ethyl esters XIV, XXV and XXXVII. Compounds XXIV, XXV, XXXVI and XXXVII were converted into the corresponding hemisuccinates XXVIII, XXIX, XL and XLI and β-D-glucosides XXXII, XXXIII, XLIV and XLV.
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TURCAN, Doina, Lucia ANDRIES, Alexandr DORIF, and Victoria SACARA. "Analysis of clinical and molecular genetic characteristics of Wiskott-Aldrich syndrome and X-linked thrombocytopenia." One Health & Risk Management 2, no. 3 (June 17, 2021): 61–66. http://dx.doi.org/10.38045/ohrm.2021.3.10.

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Introduction. Wiskott-Aldrich syndrome is a rare X-linked disorder characterized by microthrombocytopenia, eczema, and recurrent infections. It is caused by mutations of the WAS gene which encodes the WAS protein (WASp) – a key regulator of actin polymerization in hematopoietic cells. Mutations within the WASp gene result in a wide heterogeneity of clinical disease, ranging from ‘classical WAS’ to mild asymptomatic thrombocytopenia (X-linked thrombocytopenia [XLT]), or congenital neutropenia (X-lined neutropenia [XLN]).Case presentation. This present paper reports a phenotypical and laboratory
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6

Luce, T. J., and John Briscoe. "Livius, Ab Urbe Condita libri XLI-XLV." American Journal of Philology 109, no. 3 (1988): 455. http://dx.doi.org/10.2307/294903.

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7

Summers, G. D. "An aerial Survey of Çevre Kale, Yaraşli." Anatolian Studies 42 (December 1992): 179–206. http://dx.doi.org/10.2307/3642957.

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About 1 km. north of the village of Yaraşlı on a natural hill that forms an extension of Karaca Daǧ, is a large and impressively defended site locally called Çevre Kale (Fig. 1). Pottery from the surface is Imperial Hittite and Phrygian in date. There is later material, Hellenistic to Byzantine, beneath and adjacent to the village. Yaraşlı is a large well watered village in the Kulu district (ilce) of Konya province (il). The map reference is 59-Ie on the 1:200,000 sheet for Katrancı (Harita Genel Müdürlüǧü 1945).The aims of the project were to produce a photographic record of the site from th
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8

Hannan, Khyle M. "Book Review: Encyclopedia of African American Business: Updated and Revised Edition, 2nd ed." Reference & User Services Quarterly 58, no. 1 (October 10, 2018): 62. http://dx.doi.org/10.5860/rusq.58.1.6853.

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The African American contribution to business and economic institutions in America is significant and spans “the period from 18th-century America to the present” (xlv). This encyclopedia is unique in being a reference work dedicated solely to exploring this contribution and its impact. In the preface, the editor, Jessie Carney Smith, Dean of the Library and Camille Cosby Distinguished Chair in the Humanities at Fisk University in Nashville, TN, mentions, “one subject that has been met with somewhat limited appeal is African American books on businesses, merely because the focus is narrow and u
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9

Blundell, Michael P., Austen Worth, Gerben Bouma, and Adrian J. Thrasher. "The Wiskott-Aldrich Syndrome: The Actin Cytoskeleton and Immune Cell Function." Disease Markers 29, no. 3-4 (2010): 157–75. http://dx.doi.org/10.1155/2010/781523.

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Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive primary immunodeficiency characterised by immune dysregulation, microthrombocytopaenia, eczema and lymphoid malignancies. Mutations in the WAS gene can lead to distinct syndrome variations which largely, although not exclusively, depend upon the mutation. Premature termination and deletions abrogate Wiskott-Aldrich syndrome protein (WASp) expression and lead to severe disease (WAS). Missense mutations usually result in reduced protein expression and the phenotypically milder X-linked thrombocytopenia (XLT) or attenuated WAS [1-3]. Mor
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10

Thrasher, Adrian J. "New insights into the biology of Wiskott-Aldrich syndrome (WAS)." Hematology 2009, no. 1 (January 1, 2009): 132–38. http://dx.doi.org/10.1182/asheducation-2009.1.132.

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Abstract The Wiskott-Aldrich syndrome (WAS) is an X-linked immunodeficiency disease with a characteristic clinical phenotype that includes thrombocytopenia with small platelets, eczema, recurrent infections due to immunodeficiency, and an increased incidence of autoimmune manifestations and malignancies. The identification of the molecular defect in the WAS gene has broadened the clinical spectrum of disease to include chronic or intermittent X-linked thrombocytopenia (XLT), a relatively mild form of WAS, and X-linked neutropenia (XLN) due to an arrest of myelopoiesis. The pathophysiological m
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11

Brenišínová, Monika. "Bibliografía de Ibero-Americana Pragensia, XLI-XLV, 2007-2017." IBERO-AMERICANA PRAGENSIA 45, no. 2 (May 29, 2018): 185–92. http://dx.doi.org/10.14712/24647063.2017.22.

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12

Schmid, Jana Pachlopnik, Danielle Canioni, Despina Moshous, Fabien Touzot, Nizar Mahlaoui, Fabian Hauck, Hirokazu Kanegane, et al. "Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency)." Blood 117, no. 5 (February 3, 2011): 1522–29. http://dx.doi.org/10.1182/blood-2010-07-298372.

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Abstract X-linked lymphoproliferative syndromes (XLP) are primary immunodeficiencies characterized by a particular vulnerability toward Epstein-Barr virus infection, frequently resulting in hemophagocytic lymphohistiocytosis (HLH). XLP type 1 (XLP-1) is caused by mutations in the gene SH2D1A (also named SAP), whereas mutations in the gene XIAP underlie XLP type 2 (XLP-2). Here, a comparison of the clinical phenotypes associated with XLP-1 and XLP-2 was performed in cohorts of 33 and 30 patients, respectively. HLH (XLP-1, 55%; XLP-2, 76%) and hypogammaglobulinemia (XLP-1, 67%; XLP-2, 33%) occur
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13

Roy, Sunetra, Abinadabe J. de Melo, Yao Xu, Satish K. Tadi, Aurélie Négrel, Eric Hendrickson, Mauro Modesti, and Katheryn Meek. "XRCC4/XLF Interaction Is Variably Required for DNA Repair and Is Not Required for Ligase IV Stimulation." Molecular and Cellular Biology 35, no. 17 (June 22, 2015): 3017–28. http://dx.doi.org/10.1128/mcb.01503-14.

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The classic nonhomologous end-joining (c-NHEJ) pathway is largely responsible for repairing double-strand breaks (DSBs) in mammalian cells. XLF stimulates the XRCC4/DNA ligase IV complex by an unknown mechanism. XLF interacts with XRCC4 to form filaments of alternating XRCC4 and XLF dimers that bridge DNA endsin vitro, providing a mechanism by which XLF might stimulate ligation. Here, we characterize two XLF mutants that do not interact with XRCC4 and cannot form filaments or bridge DNAin vitro. One mutant is fully sufficient in stimulating ligation by XRCC4/Lig4in vitro; the other is not. Thi
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14

Musilli, Stefania, Vincent Abramowski, Benoit Roch, and Jean-Pierre de Villartay. "An in vivo study of the impact of deficiency in the DNA repair proteins PAXX and XLF on development and maturation of the hemolymphoid system." Journal of Biological Chemistry 295, no. 8 (January 8, 2020): 2398–406. http://dx.doi.org/10.1074/jbc.ac119.010924.

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Repair of DNA double-strand breaks by the nonhomologous end joining pathway is central for proper development of the adaptive immune system. This repair pathway involves eight factors, including XRCC4-like factor (XLF)/Cernunnos and the paralog of XRCC4 and XLF, PAXX nonhomologous end joining factor (PAXX). Xlf−/− and Paxx−/− mice are viable and exhibit only a mild immunophenotype. However, mice lacking both PAXX and XLF are embryonic lethal because postmitotic neurons undergo massive apoptosis in embryos. To decipher the roles of PAXX and XLF in both variable, diversity, and joining recombina
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15

Westerberg, Lisa S., Parool Meelu, Marisa Baptista, Michelle A. Eston, David A. Adamovich, Vinicius Cotta-de-Almeida, Brian Seed, et al. "Activating WASP mutations associated with X-linked neutropenia result in enhanced actin polymerization, altered cytoskeletal responses, and genomic instability in lymphocytes." Journal of Experimental Medicine 207, no. 6 (May 31, 2010): 1145–52. http://dx.doi.org/10.1084/jem.20091245.

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X-linked neutropenia (XLN) is caused by activating mutations in the Wiskott-Aldrich syndrome protein (WASP) that result in aberrant autoinhibition. Although patients with XLN appear to have only defects in myeloid lineages, we hypothesized that activating mutations of WASP are likely to affect the immune system more broadly. We generated mouse models to assess the role of activating WASP mutations associated with XLN (XLN-WASP) in lymphocytes. XLN-WASP is expressed stably in B and T cells and induces a marked increase in polymerized actin. XLN-WASP–expressing B and T cells migrate toward chemo
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16

Mannhalter, Christine, M. Fischer, and W.-M. Halbmayer. "Faktor-XII-Mangel und Thrombophilie." Hämostaseologie 13, no. 04 (July 1993): 157–60. http://dx.doi.org/10.1055/s-0038-1655230.

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ZusammenfassungDer F-Xll-Mangel wird autosomal rezessiv vererbt und existiert als quantitativer oder qualitativer, dysfunktioneller Mangel. Milde (heterozygote) F-Xll-Mängel weisen meist eine nur geringfügige oder sogar keine Verlängerung der aPTT auf und können somit leicht übersehen werden. Obwohl schwere (homozygote) F-Xll-Mängel deutlich verlängerte aPTT-Zeiten zeigen, wird in Zusammenhang mit dem F-Xll-Mangel üblicherweise keine Blutungsneigung beobachtet, sondern die Patienten neigen im Gegenteil - wie auch John Hageman - zu thromboembolischen Geschehen. Bei Patienten mit rezidivierenden
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17

Gew, Lai Ti, and Misni Misran. "Mixed Langmuir Monolayers of C18 Fatty Acids: Effect of Degree of Saturation." Materials Science Forum 990 (May 2020): 117–23. http://dx.doi.org/10.4028/www.scientific.net/msf.990.117.

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The degree of unsaturation of hydrocarbon chains in the lipid diversity of biological membrane will greatly influence the membrane protein function. Lipid-lipid interactions in the membrane domain are crucial in the development of novel liposomal nanocarrier as the stability and function of liposomes are important points to be considered. The embedded therapeutic proteins are dependent on lipid membrane composition and their physical properties. In this study, we elucidated lipid-lipid interactions in the biological membrane domain with energetic quantitative data by mixing any two C18 fatty a
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18

Gago-Fuentes, Raquel, and Valentyn Oksenych. "Non-Homologous End Joining Factors XLF, PAXX and DNA-PKcs Maintain the Neural Stem and Progenitor Cell Population." Biomolecules 11, no. 1 (December 28, 2020): 20. http://dx.doi.org/10.3390/biom11010020.

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Non-homologous end-joining (NHEJ) is a major DNA repair pathway in mammalian cells that recognizes, processes and fixes DNA damage throughout the cell cycle and is specifically important for homeostasis of post-mitotic neurons and developing lymphocytes. Neuronal apoptosis increases in the mice lacking NHEJ factors Ku70 and Ku80. Inactivation of other NHEJ genes, either Xrcc4 or Lig4, leads to massive neuronal apoptosis in the central nervous system (CNS) that correlates with embryonic lethality in mice. Inactivation of either Paxx, Mri or Dna-pkcs NHEJ gene results in normal CNS development d
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19

Avagyan, Serine, Michael Churchill, Kenta Yamamoto, Jennifer L. Crowe, Chen Li, Brian J. Lee, Tian Zheng, Siddhartha Mukherjee, and Shan Zha. "Hematopoietic stem cell dysfunction underlies the progressive lymphocytopenia in XLF/Cernunnos deficiency." Blood 124, no. 10 (September 4, 2014): 1622–25. http://dx.doi.org/10.1182/blood-2014-05-574863.

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Key Points XLF-deficient mice recapitulate the lymphocytopenia of XLF-deficient patients. Premature aging of hematopoietic stem cells underlies the severe and progressive lymphocytopenia in XLF-deficient mice.
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20

Chen, Bo-Ruei, Annabel Quinet, Andrea K. Byrum, Jessica Jackson, Matteo Berti, Saravanabhavan Thangavel, Andrea L. Bredemeyer, et al. "XLF and H2AX function in series to promote replication fork stability." Journal of Cell Biology 218, no. 7 (May 23, 2019): 2113–23. http://dx.doi.org/10.1083/jcb.201808134.

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XRCC4-like factor (XLF) is a non-homologous end joining (NHEJ) DNA double strand break repair protein. However, XLF deficiency leads to phenotypes in mice and humans that are not necessarily consistent with an isolated defect in NHEJ. Here we show that XLF functions during DNA replication. XLF undergoes cell division cycle 7–dependent phosphorylation; associates with the replication factor C complex, a critical component of the replisome; and is found at replication forks. XLF deficiency leads to defects in replication fork progression and an increase in fork reversal. The additional loss of H
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Gerrits, Anja J., Emily Leven, Andrew L. Frelinger, Michelle A. Berny-Lang, Hannah Tamary, Shoshana Revel-Vilk, Sabrina L. Carmichael, W. Beau Mitchell, Alan D. Michelson, and James B. Bussel. "Effects Of Eltrombopag On Thrombocytopenia, Platelet Function and Bleeding In Patients With Wiskott-Aldrich Syndrome/X-Linked Thrombocytopenia." Blood 122, no. 21 (November 15, 2013): 3536. http://dx.doi.org/10.1182/blood.v122.21.3536.3536.

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Abstract Introduction Patients with Wiskott-Aldrich syndrome (WAS) including X-linked thrombocytopenia (XLT) have microthrombocytopenia, and hemorrhage is a major problem. Current management options in WAS/XLT patients include splenectomy, human stem cell transplant (HSCT) and gene therapy. In this study, we asked whether eltrombopag, a thrombopoietin mimetic, would increase platelet counts, improve platelet function, and/or reduce bleeding in WAS/XLT patients. Methods In 9 WAS/XLT patients and 8 age-matched healthy control subjects, flow cytometry was used to assess platelet function by surfa
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Veilleux, Louis-Nicolas, Moira S. Cheung, Francis H. Glorieux, and Frank Rauch. "The Muscle-Bone Relationship in X-Linked Hypophosphatemic Rickets." Journal of Clinical Endocrinology & Metabolism 98, no. 5 (May 1, 2013): E990—E995. http://dx.doi.org/10.1210/jc.2012-4146.

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Context: We recently found that patients with X-linked hypophosphatemic rickets (XLH) have a muscle function deficit in the lower extremities. As muscle force and bone mass are usually closely related, we hypothesized that patients with XLH could also have a bone mass deficit in the lower extremities. Objective: The study objective was to assess the muscle-bone relationship in the lower extremities of patients with XLH. Setting: The study was carried out in the outpatients department of a pediatric orthopedic hospital. Patients and Other Participants: Thirty individuals with XLH (6 to 60 y; 9
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Sharifi, Reza, Joanna C. Sinclair, Kimberly C. Gilmour, Peter D. Arkwright, Christine Kinnon, Adrian J. Thrasher, and H. Bobby Gaspar. "SAP mediates specific cytotoxic T-cell functions in X-linked lymphoproliferative disease." Blood 103, no. 10 (May 15, 2004): 3821–27. http://dx.doi.org/10.1182/blood-2003-09-3359.

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Abstract Cytotoxic T cells (CTLs) and natural killer cells play a major role in the immune response to Epstein-Barr virus (EBV) infection. In X-linked lymphoproliferative (XLP) disease, a severe immunodeficiency, immunodysregulatory phenomena are observed following EBV infection, suggesting that defects exist in these effector populations. The gene defective in XLP is SAP (signaling lymphocytic activation molecule [SLAM]–associated protein), an adaptor protein that mediates signals through SLAM and other immunoglobulin superfamily receptors including 2B4. We generated EBV-specific T-cell lines
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Gontcharenko, Victoria E., Mikhail A. Kiskin, Vladimir D. Dolzhenko, Vladislav M. Korshunov, Ilya V. Taydakov, and Yury A. Belousov. "Mono- and Mixed Metal Complexes of Eu3+, Gd3+, and Tb3+ with a Diketone, Bearing Pyrazole Moiety and CHF2-Group: Structure, Color Tuning, and Kinetics of Energy Transfer between Lanthanide Ions." Molecules 26, no. 9 (May 1, 2021): 2655. http://dx.doi.org/10.3390/molecules26092655.

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Three novel lanthanide complexes with the ligand 4,4-difluoro-1-(1,5-dimethyl-1H-pyrazol-4-yl)butane-1,3-dione (HL), namely [LnL3(H2O)2], Ln = Eu, Gd and Tb, were synthesized, and, according to single-crystal X-ray diffraction, are isostructural. The photoluminescent properties of these compounds, as well as of three series of mixed metal complexes [EuxTb1-xL3(H2O)2] (EuxTb1-xL3), [EuxGd1-xL3(H2O)2] (EuxGd1-xL3), and [GdxTb1-xL3(H2O)2] (GdxTb1-xL3), were studied. The EuxTb1-xL3 complexes exhibit the simultaneous emission of both Eu3+ and Tb3+ ions, and the luminescence color rapidly changes fr
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Miller, Kyle, Michael Silvey, Derek Logsdon, Frederick Balch, Ndona Nsumu, Inna Sokolovsky, Margaret Gibson, Chengpeng Bi, Daniel P. Heruth, and Robert A. White. "The Xla (X-linked anemia) Mouse: A Transient Neonatal Anemia Caused by a Gata1 Splicing Mutation,." Blood 118, no. 21 (November 18, 2011): 3162. http://dx.doi.org/10.1182/blood.v118.21.3162.3162.

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Abstract Abstract 3162 The Xla (X-linked anemia) mutant mouse was generated by N-ethyl-N-nitrosourea (ENU) mutagenesis and results in a severe and transient neonatal anemia. Xla/+ females exhibit severe anemia with 50% the level of red blood cell number, hematocrit and hemoglobin. Male Xla mice die in utero at 10.5 days gestation. The neonatal anemia observed in Xla/+ female pups is resolved by weaning age at 3 weeks by which time the mice present with a normal hematological phenotype. It is unknown how the neonatal anemia in Xla/+ females is alleviated. Previously, we mapped the Xla locus to
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Sankararaman, Senthilkumar, Rosario Maria Riel-Romero, Majed Jeroudi, and Eduardo Gonzalez-Toledo. "Epstein-Barr virus induced hemophagocytic lymphohistiocytosis in X-linked lymphoproliferative disease." Journal of Neurosciences in Rural Practice 5, no. 02 (April 2014): 171–74. http://dx.doi.org/10.4103/0976-3147.131669.

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ABSTRACTX-linked lymphoproliferative disease (XLP) is a rare, often fatal genetic disorder characterized by extreme vulnerability to Epstein-Barr virus (EBV). EBV-induced hemophagocytic lymphohistiocytosis (HLH) is a known presentation in XLP. In EBV-induced HLH in XLP, the brain imaging findings in the acute phase include a non specific pattern. In this report, we highlight the magnetic resonance imaging and magnetic resonance spectroscopy findings in a child with EBV induced HLH in XLP.
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Xue, Pu, J. P. Wang, and Xiao Ming Tao. "Strain Sensing Behaviour of PPy-Coated XLA Fibers." Applied Mechanics and Materials 142 (November 2011): 125–28. http://dx.doi.org/10.4028/www.scientific.net/amm.142.125.

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This study developed an electrically conductive XLA fiber, which can sense strain variation under extension. The conductive XLA fiber was prepared by chemical vapor deposition (CVD) after plasma surface treatment. The strain sensing behavior of the PPy-coated XLA fibers was studied under tensile loading. It is found that the sensitivity of XLA fiber bases is over 145, and its working range is from 10% to 110%, showing it excellent in strain sensing behavior.
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Zhukouskaya, Volha V., Anya Rothenbuhler, Annamaria Colao, Carolina Di Somma, Peter Kamenický, Séverine Trabado, Dominique Prié, et al. "Increased prevalence of overweight and obesity in children with X-linked hypophosphatemia." Endocrine Connections 9, no. 2 (February 2020): 144–53. http://dx.doi.org/10.1530/ec-19-0481.

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Background/aim X-linked hypophosphatemia (XLH) is a rare disease characterized by low phosphate levels. Scientific evidence points to a link between hypophosphatemia and obesity in general population. The aim of our longitudinal observational study was to investigate the prevalence of obesity and associated factors in a large cohort of children with XLH. Patients/methods We studied 172 XLH-children 5–20 years of age (113 girls/59 boys). Anthropometric parameters (weight, height, and BMI) were collected at birth and during follow-up at mean ages of 5.3, 8.2, 11.3, and 15.9 years (groups 1, 2, 3
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Ozturk, Can, Sumer Sutcuoglu, Berna Atabay, and Afig Berdeli. "X-Linked Agammaglobulinemia Presenting with Secondary Hemophagocytic Syndrome: A Case Report." Case Reports in Medicine 2013 (2013): 1–4. http://dx.doi.org/10.1155/2013/742795.

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Introduction. Coincidence of X-linked agammaglobulinemia (XLA) and secondary hemophagocytic syndrome (sHS) is atypical. Both diseases are rare and pathogenesis of the latter one is not clearly known.Case Presentation. A 5-year-old boy was diagnosed both with XLA and sHS. However, in his history, he did not have severe and recurrent infections. Bruton tyrosine kinase (BTK) gene mutation was present (c.1581_1584delTTTG). To the best of the authors’ knowledge, coincidence of XLA and sHS had not been reported in the literature before.Conclusion. Patients with XLA are extremely vulnerable to recurr
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Zhu, Zhi Wen, Xin You Huang, Hai Tang Hua, and Yan Li. "Study on the Property of Ba0.85Ca0.15Ti0.9Zr0.1O3 Lead-Free Piezoelectric Ceramics Doped with Li2CO3." Advanced Materials Research 549 (July 2012): 651–54. http://dx.doi.org/10.4028/www.scientific.net/amr.549.651.

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Ba0.85Ca0.15Ti0.9Zr0.1O3-xLi2CO3 lead-free piezoelectric ceramics (abbreviated as BCZT-xLi) were prepared by conventional solid state reaction method. The microstructure, piezoelectric properties and dielectric properties were studied for BCZT-xLi samples doped with different Li2CO3 content(x= 0, 0.05, 0.1 0.2 0.3, 0.4wt. %). The results show that the piezoelectric constant(d33), planar electron mechanical coupling coefficient(kp) and thickness electron mechanical(kt) of BCZ-T-xLi ceramics increase firstly and decreases subsequently with increasing of Li+ doping amount, the dielectric loss(tan
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Simpson, Christine A., Anna Maria Santoro, Thomas O. Carpenter, and Karl Leonard Insogna. "Serum Levels of Lipocalin Are Lower in Adolescents With X-Linked Hypophosphatemia." Journal of the Endocrine Society 5, Supplement_1 (May 1, 2021): A27. http://dx.doi.org/10.1210/jendso/bvab048.052.

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Abstract Individuals with X-linked hypophosphatemia (XLH) are at greater risk for being overweight or obese. It has generally been assumed that the primary reason for this is impaired mobility due to accelerated osteoarthritis, abnormal biomechanics of ambulation, pseudofractures and enthesopathy. These known complications limit the ability of patients with XLH to engage in regular aerobic exercise. Whether there are underlying metabolic abnormalities that also put patients with XLH at greater risk for excessive weight gain is largely unknown. A recent French study1 confirmed that patients wit
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Mao, Meng, Thomas O. Carpenter, Michael P. Whyte, Alison Skrinar, Chao-Yin Chen, Javier San Martin, and Alan D. Rogol. "Growth Curves for Children with X-linked Hypophosphatemia." Journal of Clinical Endocrinology & Metabolism 105, no. 10 (July 28, 2020): 3243–49. http://dx.doi.org/10.1210/clinem/dgaa495.

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Abstract Context We characterized linear growth in infants and children with X-linked hypophosphatemia (XLH). Objective Provide linear growth curves for children with XLH from birth to early adolescence. Design Data from 4 prior studies of XLH were pooled to construct growth curves. UX023-CL002 was an observational, retrospective chart review. Pretreatment data were collected from 3 interventional trials: two phase 2 trials (UX023-CL201, UX023-CL205) and a phase 3 trial (UX023-CL301). Setting Medical centers with expertise in treating XLH. Patients Children with XLH, 1-14 years of age. Interve
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Tabata, Yasuhiro, Joyce Villanueva, Susan M. Lee, Kejian Zhang, Hirokazu Kanegane, Toshio Miyawaki, Janos Sumegi, and Alexandra H. Filipovich. "Rapid Detection of SAP Deficiency in Cytotoxic Lymphocytes from Patients with X-Linked Lymphoproliferative Disease and Their Family Members." Blood 104, no. 11 (November 16, 2004): 3846. http://dx.doi.org/10.1182/blood.v104.11.3846.3846.

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Abstract Background: X-linked lymphoproliferative disease (XLP) is a primary immunodeficiency, which is characterized by an extreme susceptibility to Epstein-Barr virus (EBV). In half of the XLP patients, primary EBV infection can be fatal with explosive activation and proliferation of lymphocytes in many organs, which leads to fluminant hepatitis and bone marrow failure with hemophagocytosis. Mutations in the SH2D1A gene, which encodes the SAP protein, have been described in a proportion of patients with the clinical syndrome of XLP. The diagnosis of XLP is still difficult given its clinical
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34

Wohlfarth, Ariane, Shaokun Pang, Mingshe Zhu, Adarsh S. Gandhi, Karl B. Scheidweiler, Hua-fen Liu, and Marilyn A. Huestis. "First Metabolic Profile of XLR-11, a Novel Synthetic Cannabinoid, Obtained by Using Human Hepatocytes and High-Resolution Mass Spectrometry." Clinical Chemistry 59, no. 11 (November 1, 2013): 1638–48. http://dx.doi.org/10.1373/clinchem.2013.209965.

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BACKGROUND Since the mid-2000s synthetic cannabinoids have been abused as recreational drugs, prompting scheduling of these substances in many countries. To circumvent legislation, manufacturers constantly market new compounds; [1-(5-fluoropentyl)indol-3-yl]-(2,2,3,3-tetramethylcyclopropyl)methanone (XLR-11), the fluorinated UR-144 analog, is one of the most recent and widely abused drugs, and its use is now linked with acute kidney injury. Our goal was to investigate XLR-11 metabolism for identification of major urinary targets in analytical methods and to clarify the origin of metabolites wh
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Wang, Huanan, Bing Mao, and Chang Chen. "Xiaoqinglong Decoction Attenuates Chronic Obstructive Pulmonary Disease in Rats via Inhibition of Autophagy." Evidence-Based Complementary and Alternative Medicine 2018 (2018): 1–8. http://dx.doi.org/10.1155/2018/6705871.

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Effective treatment for chronic obstructive pulmonary disease (COPD) and knowledge of the underlying mechanism are urgently required. Xiaoqinglong decoction (XQL) is widely used to treat COPD in Traditional Chinese Medicine, but the mechanism remains unclear. In this study, we tested the hypothesis that XQL ameliorates COPD via inhibition of autophagy in lung tissue on a rat model. Rats were divided into five groups, namely, Control group, COPD group, COPD + XQL group, COPD + Rapamycin group, and COPD + XQL + Rapamycin group. Pathological changes on cellular and molecular levels, apoptosis ref
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36

Kelly, Sinead, Martin Cormican, Leanne Parke, Geraldine Corbett-Feeney, and John Flynn. "Cost-Effective Methods for Isolation ofSalmonella enterica in the Clinical Laboratory." Journal of Clinical Microbiology 37, no. 10 (1999): 3369. http://dx.doi.org/10.1128/jcm.37.10.3369-3369.1999.

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Data from 8,717 fecal specimens indicate that primary inoculation of xylose lysine deoxycholate (XLD) agar may enhance the speed, but not the sensitivity, of isolation of Salmonella enterica over that achieved with Selenite enrichment only. Plating of Selenite broth onto both brilliant green and XLD agar offers no advantage over plating onto XLD alone.
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Hawley, Samuel, Nick J. Shaw, Antonella Delmestri, Daniel Prieto-Alhambra, Cyrus Cooper, Rafael Pinedo-Villanueva, and M. Kassim Javaid. "Prevalence and Mortality of Individuals With X-Linked Hypophosphatemia: A United Kingdom Real-World Data Analysis." Journal of Clinical Endocrinology & Metabolism 105, no. 3 (November 15, 2019): e871-e878. http://dx.doi.org/10.1210/clinem/dgz203.

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Abstract Background X-linked hypophosphatemia (XLH) is a rare multisystemic disease with a prominent musculoskeletal phenotype. We aim here to improve understanding of the prevalence of XLH across the life course and of overall survival among people with XLH. Methods This was a population-based cohort study using a large primary care database in the United Kingdom (UK) from 1995 to 2016. XLH cases were matched by age, gender, and practice to up to 4 controls. Trends in prevalence over the study period were estimated (stratified by age) and survival among cases was compared with that of control
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Baroncelli, Giampiero I., Silvano Bertelloni, Mirna Cosci o Di Coscio, Nina Tyutyusheva, Sofia D’Elios, and Diego Peroni. "Management of patients with X-linked hypophosphatemic rickets during Covid-19 pandemic lockdown." Journal of Pediatric Endocrinology and Metabolism 34, no. 7 (April 23, 2021): 905–10. http://dx.doi.org/10.1515/jpem-2021-0217.

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Abstract Objectives To identify a safe pathway for management and treatment of patients with X-linked hypophosphatemic rickets (XLH) during Covid-19 pandemic lockdown. Methods Twenty-six patients with XLH (age 3.1–25.7 years) were enrolled in Pediatric Endocrine Unit; nine of them were receiving human monoclonal anti-fibroblast growth factor 23 antibody (burosumab) and 17 (pediatric patients, age 9.5–17.9 years, n=7; young-adult patients, age 20.1–25.7 years, n=10) received conventional treatment with inorganic oral phosphate salts and active vitamin D metabolites. A Covid-19 free pathway was
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39

Dixon, Peter H., Paul T. Christie, Carol Wooding, Dorothy Trump, Marvin Grieff, Ingrid Holm, Joseph M. Gertner, et al. "Mutational Analysis of PHEX Gene in X-Linked Hypophosphatemia1." Journal of Clinical Endocrinology & Metabolism 83, no. 10 (October 1, 1998): 3615–23. http://dx.doi.org/10.1210/jcem.83.10.5180.

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Hypophosphatemic rickets is commonly an X-linked dominant disorder (XLH or HYP) associated with a renal tubular defect in phosphate transport and bone deformities. The XLH gene, referred to as PHEX, or formerly as PEX (phosphate regulating gene with homologies to endopeptidases on the X-chromosome), encodes a 749-amino acid protein that putatively consists of an intracellular, transmembrane, and extracellular domain. PHEX mutations have been observed in XLH patients, and we have undertaken studies to characterize such mutations in 46 unrelated XLH kindreds and 22 unrelated patients with nonfam
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40

Vincze, Jon, Brian W. Skinner, Katherine A. Tucker, Kory A. Conaway, Jonathan W. Lowery, and Julia M. Hum. "The Metabolic Bone Disease X-linked Hypophosphatemia: Case Presentation, Pathophysiology and Pharmacology." Life 11, no. 6 (June 15, 2021): 563. http://dx.doi.org/10.3390/life11060563.

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The authors present a stereotypical case presentation of X-linked hypophosphatemia (XLH) and provide a review of the pathophysiology and related pharmacology of this condition, primarily focusing on the FDA-approved medication burosumab. XLH is a renal phosphate wasting disorder caused by loss of function mutations in the PHEX gene (phosphate-regulating gene with homologies to endopeptidases on the X chromosome). Typical biochemical findings include elevated serum levels of bioactive/intact fibroblast growth factor 23 (FGF23) which lead to (i) low serum phosphate levels, (ii) increased fractio
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41

Ford, P., G. Amodeo, C. Capurro, C. Ibarra, R. Dorr, P. Ripoche, and M. Parisi. "Progesterone inhibition of water permeability in Bufo arenarum oocytes and urinary bladder." American Journal of Physiology-Renal Physiology 270, no. 5 (May 1, 1996): F880—F885. http://dx.doi.org/10.1152/ajprenal.1996.270.5.f880.

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The ovarian oocytes from Bufo arenarum (BAO) but not those from Xenopus laevis (XLO) would have water channels (WC). We now report that the injection of the mRNA from BAO into the oocytes from XLO increased their water osmotic permeability (Pi) (reduced by 0.3 mM HgCl2 and reversed by 5 mM beta-mercaptoethanol). A 30-min challenge with progesterone induced, 18 h later, a reduction of the mercury-sensitive fraction of Pf in the BAO (but not in XLO). The mRNA from BAO pretreated with progesterone lost its capacity to induce WC in the XLO, but the hormone did not affect the expression of the WC i
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42

Nahum, Amit, David Manson, and Bo Ngan. "Atypical presentation and manifestations in X-linked agammaglobulinemia patients with novel BTK mutations." LymphoSign Journal 2, no. 2 (June 1, 2015): 75–83. http://dx.doi.org/10.14785/lpsn-2014-0015.

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X-linked agammaglobulinemia (XLA) is a rare immunodeficiency caused by defects in the Bruton tyrosine kinase (BTK) gene, characterized by impaired B-cell development, reduced immunoglobulin production, and increased susceptibility to bacterial infections at an early age. Some XLA patients show atypical presentations, with most reports concentrating on the diagnosis at a relatively old age. They presented with infections at late age or with unusual pathogens; however, other atypical manifestations have only rarely been reported. Methods: Description of patients with XLA and novel mutations in B
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43

Diop, S. N., M. A. Diallo, C. K. Diawara, and D. Cot. "Intrinsic properties and performances of NF270 and XLE membranes for water filtration." Water Supply 11, no. 2 (April 1, 2011): 186–93. http://dx.doi.org/10.2166/ws.2011.024.

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Nanofiltration and low pressure reverse osmosis membranes are well-known in the field of drinking water production and their separation performance is very strongly related to their intrinsic characteristics. The membrane characterization (scanning electron microscopy (SEM), atomic force microscopy (AFM), zeta potential …) was realized on a NF270 and extra-low energy (XLE) membrane. SEM results of virgin NF270 and XLE membranes show that both are about the same thickness whereas that of the active layer of NF270 membrane is weaker than that of the XLE. The AFM measurements show that the roughn
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Sochorová, Klára, Rudolf Horváth, Daniela Rožková, Jiří Litzman, Jiřina Bartůňková, Anna Šedivá та Radek Špíšek. "Impaired Toll-like receptor 8–mediated IL-6 and TNF-α production in antigen-presenting cells from patients with X-linked agammaglobulinemia". Blood 109, № 6 (7 листопада 2006): 2553–56. http://dx.doi.org/10.1182/blood-2006-07-037960.

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Abstract The critical role of Bruton tyrosine kinase (Btk) in B cells has been documented by the block of B-cell development in X-linked agammaglobulinemia (XLA). Less is known about Btk function in myeloid cells. Several pieces of evidence indicate that Btk is a component of Toll-like receptor (TLR) signaling. We analyzed whether Btk deficiency in XLA is associated with an impaired dendritic cell (DC) compartment or defective TLR signaling. We analyzed the expression of TLRs 1 to 9 on myeloid DCs generated from XLA patients and evaluated their response to activation by specific TLR agonists.
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45

Wu, Qian, Takashi Ochi, Dijana Matak-Vinkovic, Carol V. Robinson, Dimitri Y. Chirgadze, and Tom L. Blundell. "Non-homologous end-joining partners in a helical dance: structural studies of XLF–XRCC4 interactions." Biochemical Society Transactions 39, no. 5 (September 21, 2011): 1387–92. http://dx.doi.org/10.1042/bst0391387.

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XRCC4 (X-ray cross-complementation group 4) and XLF (XRCC4-like factor) are two essential interacting proteins in the human NHEJ (non-homologous end-joining) pathway that repairs DNA DSBs (double-strand breaks). The individual crystal structures show that the dimeric proteins are homologues with protomers containing head domains and helical coiled-coil tails related by approximate two-fold symmetry. Biochemical, mutagenesis, biophysical and structural studies have identified the regions of interaction between the two proteins and suggested models for the XLF–XRCC4 complex. An 8.5 Å (1 Å=0.1 nm
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Mahaney, Brandi L., Michal Hammel, Katheryn Meek, John A. Tainer, and Susan P. Lees-Miller. "XRCC4 and XLF form long helical protein filaments suitable for DNA end protection and alignment to facilitate DNA double strand break repair." Biochemistry and Cell Biology 91, no. 1 (February 2013): 31–41. http://dx.doi.org/10.1139/bcb-2012-0058.

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DNA double strand breaks (DSBs), induced by ionizing radiation (IR) and endogenous stress including replication failure, are the most cytotoxic form of DNA damage. In human cells, most IR-induced DSBs are repaired by the nonhomologous end joining (NHEJ) pathway. One of the most critical steps in NHEJ is ligation of DNA ends by DNA ligase IV (LIG4), which interacts with, and is stabilized by, the scaffolding protein X-ray cross-complementing gene 4 (XRCC4). XRCC4 also interacts with XRCC4-like factor (XLF, also called Cernunnos); yet, XLF has been one of the least mechanistically understood pro
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Tabata, Yasuhiro, Joyce Villanueva, Susan Molleran Lee, Kejian Zhang, Hirokazu Kanegane, Toshio Miyawaki, Janos Sumegi, and Alexandra H. Filipovich. "Rapid detection of intracellular SH2D1A protein in cytotoxic lymphocytes from patients with X-linked lymphoproliferative disease and their family members." Blood 105, no. 8 (April 15, 2005): 3066–71. http://dx.doi.org/10.1182/blood-2004-09-3651.

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AbstractMutations in the SH2D1A gene have been described in most patients with the clinical syndrome of X-linked lymphoproliferative disease (XLP). The diagnosis of XLP is still difficult given its clinical heterogeneity and the lack of a readily available rapid diagnostic laboratory test, particularly in patients without a family history of XLP. XLP should always be a consideration in males with Epstein-Barr virus–associated hemophagocytic lymphohistiocytosis (EBV-HLH). Four-color flow cytometric analysis was used to establish normal patterns of SH2D1A protein expression in lymphocyte subsets
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Hao, Yongbiao, Kazuko Nagase, Kazutoshi Hori, Shenglan Wang, Yoko Kogure, Ken Fukunaga, Shinichiro Kashiwamura, et al. "Xilei San Ameliorates Experimental Colitis in Rats by Selectively Degrading Proinflammatory Mediators and Promoting Mucosal Repair." Evidence-Based Complementary and Alternative Medicine 2014 (2014): 1–10. http://dx.doi.org/10.1155/2014/569587.

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Xilei san (XLS), a herbal preparation widely used in China for erosive and ulcerative diseases, has been shown to be effective in ulcerative colitis (UC). The present experiments were conducted to assess its efficacy and determine its mechanism of action in a rat model that resembles human UC. The model was induced by adding 4% dextran sulfate sodium (DSS) to the rats’ drinking water for 7 days. XLS was administered daily by retention enema from day 2 to day 7; the rats were sacrificed on day 8. The colon tissues were obtained for further experiments. A histological damage score and the activi
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ZHANG, GUODONG, and KEITH A. LAMPEL. "Comparison of Chromogenic Biolog Rainbow Agar Shigella/Aeromonas with Xylose Lysine Desoxycholate Agar for Isolation and Detection of Shigella spp. from Foods†." Journal of Food Protection 73, no. 8 (August 1, 2010): 1458–65. http://dx.doi.org/10.4315/0362-028x-73.8.1458.

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Shigella outbreaks are widely reported throughout the world. However, it remains a challenge to isolate Shigella spp. from foods by using conventional microbiological media. The main objective of this study was to determine the effectiveness of a novel chromogenic medium, Rainbow agar Shigella/Aeromonas (Rainbow agar), for the isolation and detection of Shigella spp. in foods. All four Shigella species, S. sonnei, S. flexneri, S. dysenteriae, and S. boydii, were studied. Rainbow agar was compared with tryptic soy agar, xylose lysine desoxycholate agar (XLD), and Salmonella Shigella agar (SSA)
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Amenta, Eva, Helen E. King, Holger Petermann, Vuk Uskoković, Steven M. Tommasini, and Carolyn M. Macica. "Vibrational spectroscopic analysis of hydroxyapatite in HYP mice and individuals with X-linked hypophosphatemia." Therapeutic Advances in Chronic Disease 9, no. 12 (October 11, 2018): 268–81. http://dx.doi.org/10.1177/2040622318804753.

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Background: X-linked hypophosphatemia (XLH) is the most common form of familial phosphate-wasting disorders, due to an inactivating mutation in the phosphate-regulating neutral endopeptidase, X-linked gene. Persistent osteomalacia, enthesophytes, osteophytes, degenerative arthritis and dental abscesses/periodontal disease dominate the adult disorder. However, the impact of insufficient phosphate on hydroxyapatite composition, the major inorganic component of bone and teeth, is unknown in individuals with XLH. Methods: Using Raman spectroscopy, the carbonate (CO32−) to phosphate (PO43−) ion rat
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