Bibliografías temáticas / Beta-glucocerebrosidase

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Literatura académica sobre el tema "Beta-glucocerebrosidase"

Autor: Grafiati
Publicado: 4 de junio de 2021
Última modificación: 27 de julio de 2025

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Artículos de revistas sobre el tema "Beta-glucocerebrosidase"

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Vanderjagt, D. J., D. E. Fry, and R. H. Glew. "Human glucocerebrosidase catalyses transglucosylation between glucocerebroside and retinol." Biochemical Journal 300, no. 2 (1994): 309–15. http://dx.doi.org/10.1042/bj3000309.

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The basal activity of human placental glucocerebrosidase is elevated 16-fold by n-pentanol when assayed using p-nitrophenyl beta-D-glucopyranoside (pNPGlc) as the beta-glucosidase substrate. This enhancement of activity is the result of the formation of a transglucosylation product, n-pentyl beta-D-glucoside, in rate-determining competition with the hydrolytic reaction. The transglucosylation product accounts for approximately 80% of the reaction product generated in the presence of n-pentanol (0.18 M) when either glucocerebroside or pNPGlc was used as the substrate. This stimulatory effect ca
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2

Gopalan, V., L. B. Daniels, R. H. Glew та M. Claeyssens. "Kinetic analysis of the interaction of alkyl glycosides with two human β-glucosidases". Biochemical Journal 262, № 2 (1989): 541–48. http://dx.doi.org/10.1042/bj2620541.

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This paper addresses the similarities and differences in the topology of the catalytic centres of human liver cytosolic beta-glucosidase and placental lysosomal glucocerebrosidase, and utilizes well-documented reversible active-site-directed inhibitors. This comparative kinetic study was performed mainly to decipher the chemical and structural nature of the active site of the cytosolic beta-glucosidase, whose physiological function is unknown. Specifically, analysis of the effects of a family of alkyl beta-glucosides consistently displayed 100-250-fold lower inhibition constants with the cytos
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3

Jin, K., Y. Higaki, Y. Takagi, et al. "Analysis of beta-glucocerebrosidase and ceramidase activities in atopic and aged dry skin." Acta Dermato-Venereologica 74, no. 5 (1994): 337–40. http://dx.doi.org/10.2340/0001555574341343.

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To elucidate the mechanisms that are involved in the decrease of ceramide levels in atopic dry skin and in aged skin, we examined both the activities of beta-glucocerebrosidase, which is a major enzyme in ceramide production, and of ceramidase, which is an essential enzyme in ceramide degradation, in the stratum corneum of atopic dry skin and aged skin. The specimens of the stratum corneum of forearm skin were obtained by tape-stripping from 61 healthy volunteers and 23 patients with atopic uninvolved skin. The beta-glucocerebrosidase activity in the stratum corneum extracts was estimated usin
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Prence, E. M., K. O. Garrett, and R. H. Glew. "A kinetic study of the effects of galactocerebroside 3-sulphate on human spleen glucocerebrosidase. Evidence for two activator-binding sites." Biochemical Journal 237, no. 3 (1986): 655–62. http://dx.doi.org/10.1042/bj2370655.

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Extraction of control human spleen glucocerebrosidase with sodium cholate and butan-l-ol reversibly inactivates the enzyme in terms of its ability to hydrolyse the water-soluble substrate 4-methylumbelliferyl beta-D-glucopyranoside (MUGlc). The acidic brain lipid galactocerebroside 3-sulphate (sulphatide) reconstitutes beta-glucosidase activity in a strongly concentration-dependent manner. In this study we show that sulphatide exhibits three critical micellar concentrations (CMCs): CMC1, 3.72 microM; CMC2, 22.6 microM; CMC3, 60.7 microM. We designate the aggregates formed at these CMCs as prim
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5

Holleran, W. M., Y. Takagi, G. K. Menon, et al. "Permeability barrier requirements regulate epidermal beta-glucocerebrosidase." Journal of Lipid Research 35, no. 5 (1994): 905–12. http://dx.doi.org/10.1016/s0022-2275(20)39184-7.

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Glew, R. H., V. Gopalan, C. A. Hubbell та ін. "2,3-di-O-tetradecyl-1-O-(β-d-glucopyranosyl)-sn-glycerol is a substrate for human glucocerebrosidase". Biochemical Journal 274, № 2 (1991): 557–63. http://dx.doi.org/10.1042/bj2740557.

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Glucocerebrosidase, the lysosomal enzyme that is deficient in patients with Gaucher's disease, hydrolyses non-physiological aryl beta-D-glucosides and glucocerebroside, its substrate in vivo. We document that 2,3,-di-O-tetradecyl-1-O-(beta-D-glucopyranosyl)-sn-glycerol (2,3,-di-14:0-beta-Glc-DAG) inhibits human placental glucocerebrosidase activity in vitro (Ki 0.18 mM), and the nature of inhibition is typical of a mixed-type pattern. Furthermore, 2,3-di-14:0-beta-Glc-DAG was shown to be an excellent substrate for the lysosomal beta-glucosidase (Km 0.15 mM; Vmax. 19.8 units/mg) when compared w
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7

Mahjouba Baiya, Hicham Yahyaoui, Imane El Khannouri, Ibtissam Mhirig, Mustapha Ait Ameur, and Mohamed Chakour. "Place of cytology in the diagnosis of Gaucher disease: About a case." GSC Advanced Research and Reviews 11, no. 1 (2022): 144–47. http://dx.doi.org/10.30574/gscarr.2022.11.1.0105.

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Gaucher disease is an autosomal recessive genetic disease caused by a deficiency in a lysosomal enzyme, beta glucocerebrosidase. This disease is characterized by deposits of glucosylceramide in liver, spleen and bone marrow cells. The presentation of MG is very heterogeneous, ranging from the asymptomatic form to the lethal form. Neurological forms (types 2 and 3) are present in only 5% of patients with MG and are less frequent than non-neurological forms (type 1). The formal diagnosis is established by measuring the activity of beta glucocerebrosidase in circulating leukocytes. The accumulati
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Mahjouba, Baiya, Yahyaoui Hicham, El Khannouri Imane, Mhirig Ibtissam, Ait Ameur Mustapha, and Chakour Mohamed. "Place of cytology in the diagnosis of Gaucher disease: About a case." GSC Advanced Research and Reviews 11, no. 1 (2022): 144–47. https://doi.org/10.5281/zenodo.6631169.

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Gaucher disease is an autosomal recessive genetic disease caused by a deficiency in a lysosomal enzyme, beta glucocerebrosidase. This disease is characterized by deposits of glucosylceramide in liver, spleen and bone marrow cells. The presentation of MG is very heterogeneous, ranging from the asymptomatic form to the lethal form. Neurological forms (types 2 and 3) are present in only 5% of patients with MG and are less frequent than non-neurological forms (type 1). The formal diagnosis is established by measuring the activity of beta glucocerebrosidase in circulating leukocytes. The accumulati
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9

Colbaugh, P. A., M. Stookey, and R. K. Draper. "Impaired lysosomes in a temperature-sensitive mutant of Chinese hamster ovary cells." Journal of Cell Biology 108, no. 6 (1989): 2211–19. http://dx.doi.org/10.1083/jcb.108.6.2211.

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We describe here the properties of a mutant of Chinese hamster ovary cells that expresses a conditional-lethal mutation affecting dense lysosomes. This mutant, termed V.24.1, is a member of the End4 complementation group of temperature-sensitive mutants selected for resistance to protein toxins (Colbaugh, P. A., C.-Y. Kao, S.-P. Shia, M. Stookey, and R. K. Draper. 1988. Somatic Cell Mol. Genet. 14:499-507). Vesicles present in postnuclear supernatants prepared from V.24.1 cells harvested at the restrictive temperature had a 50% reduction in acidification activity, assessed by the ATP-stimulate
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10

Kleinotienė, Gražina, Anna Tylki-Szymanska, and Barbara Czartoryska. "Gaucher’s Disease in Lithuania: Its Diagnosis and Treatment." Medicina 47, no. 7 (2011): 405. http://dx.doi.org/10.3390/medicina47070058.

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Gaucher’s disease is a lysosomal storage disease caused by the lack of beta-glucocerebrosidase enzyme, leading to the accumulation of glucocerebroside. Gaucher’s disease is the most frequent type of sphingolipidosis as well as the most frequent lysosomal disease. Clinically, two forms of Gaucher’s disease are defined: nonneuronopathic form, so-called type 1, characterized by hepatosplenomegaly, thrombocytopenia, anemia, and osteopenia, and neuronopathic form, known as types 2 and 3, which are also characterized by hepatosplenomegaly, hematological and bone changes; however, involvement of the
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Tesis sobre el tema "Beta-glucocerebrosidase"

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BRUNIALTI, ELECTRA ATHENA SALOME'. "BETA-GLUCOCEREBROSIDASE MEDIATES MICROGLIAL NEUROPROTECTIVE FUNCTIONS: A POSSIBLE LINK BETWEEN PARKINSON'S AND GAUCHER'S DISEASES." Doctoral thesis, Università degli Studi di Milano, 2021. http://hdl.handle.net/2434/811480.

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La degenerazione dopaminergica che caratterizza il morbo di Parkinson antecede la manifestazione dei segni clinici. Identificare gli eventi molecolari responsabili dell'insorgenza della malattia di Parkinson rappresenta un traguardo fondamentale sia per poter sviluppare marcatori diagnostici che trattamenti preventivi; tuttavia, lo studio della patogenesi è limitato dalla carenza di modelli appropriati e dalla difficoltà di studiare la malattia umana che evolve lungo un arco temporale di decenni. Le indicazioni che provengono dall’analisi dei fattori di rischio, sono in questo contesto fondame
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2

Illner, Jan. "Lysosomální dědičná onemocnění: patobiochemie Gaucherovy choroby." Master's thesis, 2011. http://www.nusl.cz/ntk/nusl-297600.

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Gaucher disease is one of the lysosomal storage disorders belonging to inherited defects of catabolism of sphingolipids. These defects are caused by mutation in genes of sphingolipid hydrolases or their protein activators. Subsequent storage of non-degraded sphingolipids leads to severe clinical phenotypes in patients. Gaucher disease is caused by deficiency of lysosomal β-glucocerebrosidase (GBA1) activity. Non-degraded glycosphingolipids are glucosylceramide (GlcCer) and glucosylpsychosine (lyso-GlcCer). Accumulation of these glycosphingolipids is related to Gaucher cells which are derived f
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3

Πολυβίου, Σταύρος. "Μελέτη της γονιδιακής μεταφοράς του γονιδίου της γλυκοκερεβροσιδάσης με ιικά οχήματα σε προγονικά αιμοποιητικά κύτταρα του ανθρώπου". Thesis, 2011. http://hdl.handle.net/10889/5667.

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Η ποσοτική ή ποιοτική ανεπάρκεια του λυσοσωματικού ενζύμου γλυκοκερεβροσιδάση οδηγεί στην παθολογία της νόσου Gaucher με κεντρικό το ρόλο της συσσώρευσης του υποστρώματός της, του γλυκοκερεβροσιδίου, στα μακροφάγα. Για περισσότερες από δύο δεκαετίες χρησιμοποιούνται γ-ρετροϊικά οχήματα στην ερευνητική προσέγγιση της διόρθωσης του ελλείμματος μέσω γονιδιακής μεταφοράς του γονιδίου της γλυκοκερεβροσιδάσης. Όπως έχει αναδειχθεί και σε κλινικό επίπεδο, είναι επιτακτική η ανάγκη για το σχεδιασμό γ ρετροϊικών οχημάτων, τα οποία θα είναι όχι μόνο αποτελεσματικά αλλά και ασφαλή, με κύριο στόχο τον περ
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Sãcultanu, Mãdãlina. "Modeling neuropathological type II Gaucher Disease using iPSCs, CRISPR/Cas9 and transcription factor-driven protocols." Master's thesis, 2020. http://hdl.handle.net/10451/48415.

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Tese de mestrado em Biologia Molecular e Genética, Universidade de Lisboa, Faculdade de Ciências, 2020<br>A Doença de Gaucher (GD) é uma patologia com transmissão hereditária autossómica recessiva causada por mutações no gene GBA1 que codifica para o enzima beta-glucocerebrosidase (GCase). Esta proteína é um enzima housekeeping nos lisossomas, responsável pela degradação de glucocerebrosidase em glicose e ceramida. Com uma incidência entre 1 em 40.000 e 1 em 60.000 nascimentos na população geral, e 1 em 800 nascimentos na população de descendência Judaica Ashkenazi, é a forma mais comum de Doe
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Lukšan, Ondřej. "Mechanismy regulace exprese genů pro ornitin transkarbamylázu a beta-glukocerebrosidázu a jejich význam v diagnostice." Doctoral thesis, 2014. http://www.nusl.cz/ntk/nusl-332352.

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5 Abstract Definitive diagnosis of inherited metabolic disorders commonly depends on the measurement of enzyme activity (which is often complicated) and/or molecular genetic testing. Yet even the standard mutation analysis can bring false negative results in the case of gross chromosomal rearrangements or incorrect regulation of gene expression due to the mutations in regulatory regions. In the present study I focused on characterization of complex mutations affecting the gene encoding ornithin transcarbamylase (OTC) followed by studies of regulatory regions of OTC and GBA (the gene encoding β
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Capítulos de libros sobre el tema "Beta-glucocerebrosidase"

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Edmunds, Tim. "Beta-Glucocerebrosidase Ceredase® and Cerezyme®." In Directory of Therapeutic Enzymes. CRC Press, 2005. http://dx.doi.org/10.1201/9781420038378.ch6.

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Strauchen, James A. "Storage Disorders." In Diagnostic Histopathology of the Bone Marrow. Oxford University PressNew York, NY, 1996. http://dx.doi.org/10.1093/oso/9780195097566.003.0023.

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Abstract The storage disorders are characterized by hereditary lysosomal enzyme deficiences with resultant abnormal accumulation of storage material in macrophages in the liver, spleen, bone marrow, and other organs. The storage disorders are categorized as lipidoses and mucopolysaccharidoses. Most are inherited as autosomal-recessive traits. The accumulation of macrophages in the bone marrow and spleen results in hematologic abnormalities; neuronopathic effects are prominent in some forms. The major storage disorders that will be considered are Gaucher’s disease, Neimann-Pick disease, the “se
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