Academic literature on the topic 'SMCR8'

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Journal articles on the topic "SMCR8"

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Tang, Dan, Jingwen Sheng, Liangting Xu, et al. "Cryo-EM structure of C9ORF72–SMCR8–WDR41 reveals the role as a GAP for Rab8a and Rab11a." Proceedings of the National Academy of Sciences 117, no. 18 (2020): 9876–83. http://dx.doi.org/10.1073/pnas.2002110117.

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A massive intronic hexanucleotide repeat (GGGGCC) expansion in C9ORF72 is a genetic origin of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Recently, C9ORF72, together with SMCR8 and WDR41, has been shown to regulate autophagy and function as Rab GEF. However, the precise function of C9ORF72 remains unclear. Here, we report the cryogenic electron microscopy (cryo-EM) structure of the human C9ORF72–SMCR8–WDR41 complex at a resolution of 3.2 Å. The structure reveals the dimeric assembly of a heterotrimer of C9ORF72–SMCR8–WDR41. Notably, the C-terminal tail of C9ORF72 and
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McAlpine, William, Lei Sun, Kuan-wen Wang, et al. "Excessive endosomal TLR signaling causes inflammatory disease in mice with defective SMCR8-WDR41-C9ORF72 complex function." Proceedings of the National Academy of Sciences 115, no. 49 (2018): E11523—E11531. http://dx.doi.org/10.1073/pnas.1814753115.

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The SMCR8-WDR41-C9ORF72 complex is a regulator of autophagy and lysosomal function. Autoimmunity and inflammatory disease have been ascribed to loss-of-function mutations of Smcr8 or C9orf72 in mice. In humans, autoimmunity has been reported to precede amyotrophic lateral sclerosis caused by mutations of C9ORF72. However, the cellular and molecular mechanisms underlying autoimmunity and inflammation caused by C9ORF72 or SMCR8 deficiencies remain unknown. Here, we show that splenomegaly, lymphadenopathy, and activated circulating T cells observed in Smcr8−/− mice were rescued by triple knockout
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Liang, Chen, Qiang Shao, Wei Zhang, et al. "Smcr8 deficiency disrupts axonal transport-dependent lysosomal function and promotes axonal swellings and gain of toxicity in C9ALS/FTD mouse models." Human Molecular Genetics 28, no. 23 (2019): 3940–53. http://dx.doi.org/10.1093/hmg/ddz230.

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Abstract G4C2 repeat expansions in an intron of C9ORF72 cause the most common familial amyotrophic lateral sclerosis and frontotemporal dementia (collectively, C9ALS/FTD). Mechanisms and mediators of C9ALS/FTD pathogenesis remain poorly understood. C9orf72 and Smcr8 form a protein complex. Here, we show that expression of Smcr8, like C9orf72, is reduced in C9ALS/FTD mouse models and patient tissues. Since Smcr8 is highly conserved between human and mouse, we evaluated the effects of Smcr8 downregulation in mice. Smcr8 knockout (KO) mice exhibited motor behavior deficits, which resemble those o
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Nörpel, Julia, Simone Cavadini, Andreas D. Schenk, et al. "Structure of the human C9orf72-SMCR8 complex reveals a multivalent protein interaction architecture." PLOS Biology 19, no. 7 (2021): e3001344. http://dx.doi.org/10.1371/journal.pbio.3001344.

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A major cause of familial amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) spectrum disorder is the hexanucleotide G4C2 repeat expansion in the first intron of the C9orf72 gene. Many underlying mechanisms lead to manifestation of disease that include toxic gain-of-function by repeat G4C2 RNAs, dipeptide repeat proteins, and a reduction of the C9orf72 gene product. The C9orf72 protein interacts with SMCR8 and WDR41 to form a trimeric complex and regulates multiple cellular pathways including autophagy. Here, we report the structure of the C9orf72-SMCR8 complex at 3.8 Å reso
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Yang, Mei, Chen Liang, Kunchithapadam Swaminathan, et al. "A C9ORF72/SMCR8-containing complex regulates ULK1 and plays a dual role in autophagy." Science Advances 2, no. 9 (2016): e1601167. http://dx.doi.org/10.1126/sciadv.1601167.

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The intronic GGGGCC hexanucleotide repeat expansion in chromosome 9 open reading frame 72 (C9ORF72) is a prevalent genetic abnormality identified in both frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). Smith-Magenis syndrome chromosomal region candidate gene 8 (SMCR8) is a protein with unclear functions. We report that C9ORF72 is a component of a multiprotein complex containing SMCR8, WDR41, and ATG101 (an important regulator of autophagy). The C9ORF72 complex displays guanosine triphosphatase (GTPase) activity and acts as a guanosine diphosphate–guanosine 5′-triphosphat
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Amick, Joseph, Agnes Roczniak-Ferguson, and Shawn M. Ferguson. "C9orf72 binds SMCR8, localizes to lysosomes, and regulates mTORC1 signaling." Molecular Biology of the Cell 27, no. 20 (2016): 3040–51. http://dx.doi.org/10.1091/mbc.e16-01-0003.

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Hexanucleotide expansion in an intron of the C9orf72 gene causes amyotrophic lateral sclerosis and frontotemporal dementia. However, beyond bioinformatics predictions that suggested structural similarity to folliculin, the Birt-Hogg-Dubé syndrome tumor suppressor, little is known about the normal functions of the C9orf72 protein. To address this problem, we used genome-editing strategies to investigate C9orf72 interactions, subcellular localization, and knockout (KO) phenotypes. We found that C9orf72 robustly interacts with SMCR8 (a protein of previously unknown function). We also observed tha
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Jung, Jennifer, and Christian Behrends. "Multifaceted role of SMCR8 as autophagy regulator." Small GTPases 11, no. 1 (2017): 53–61. http://dx.doi.org/10.1080/21541248.2017.1346553.

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Talaia, Gabriel, Joseph Amick, and Shawn M. Ferguson. "Receptor-like role for PQLC2 amino acid transporter in the lysosomal sensing of cationic amino acids." Proceedings of the National Academy of Sciences 118, no. 8 (2021): e2014941118. http://dx.doi.org/10.1073/pnas.2014941118.

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PQLC2, a lysosomal cationic amino acid transporter, also serves as a sensor that responds to scarcity of its substrates by recruiting a protein complex composed of C9orf72, SMCR8, and WDR41 to the surface of lysosomes. This protein complex controls multiple aspects of lysosome function. Although it is known that this response to changes in cationic amino acid availability depends on an interaction between PQLC2 and WDR41, the underlying mechanism for the regulated interaction is not known. In this study, we present evidence that the WDR41–PQLC2 interaction is mediated by a short peptide motif
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Zhao, Shidong, Wenwen Ru, Xinyan Chen, et al. "Structural insights into SMCR8 C-degron recognition by FEM1B." Biochemical and Biophysical Research Communications 557 (June 2021): 236–39. http://dx.doi.org/10.1016/j.bbrc.2021.04.046.

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Kumar, Vijay. "Molecular interactions between C9ORF72 and SMCR8: A local energetic frustration perspective." Biochemical and Biophysical Research Communications 570 (September 2021): 1–7. http://dx.doi.org/10.1016/j.bbrc.2021.07.016.

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Dissertations / Theses on the topic "SMCR8"

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Pietri, David. "Structure and function of the C9ORF72-SMCR8-WDR41 complex and its implication for Amyotrophic Lateral Sclerosis (ALS)." Electronic Thesis or Diss., Strasbourg, 2023. http://www.theses.fr/2023STRAJ087.

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La sclérose latérale amyotrophique (SLA ou maladie de Charcot) est la troisième maladie neurodégénérative la plus répandue. La principale cause génétique de la SLA est une expansion de répétitions GGGGCC dans le gène C9ORF72, dont la protéine forme un complexe avec les protéines SMCR8 et WDR41. Afin de mieux comprendre ses fonctions moléculaires, résoudre sa structure était un objectif principal de ma thèse. En parallèle, nous avons découvert que C9ORF72 régule un mécanisme nouvellement décrit de biogenèse de nouveaux lysosomes nommé reformation autophagique des lysosomes (ALR). Ce processus a
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O'Donohue, Daniel J. "First-term retention of enlisted Selected Marine Corps (SMCR) Reservists." Thesis, Monterey, California. Naval Postgraduate School, 1988. http://hdl.handle.net/10945/23233.

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This thesis examines factors that influence a male, first-term enlisted reservist's decision to remain in the Selected Marine Corps Reserve (SMCR). Specifically, the logistic regression model was used to determine the relative impact of bio-demographic and both pecuniary and nonpecuniary job factors on retention. Models were developed for both nonprior (NPS) and prior active service (PS) reservists. The database was a combination of the responses of participants in the 1986 Reserve Components Surveys and their personnel records from the Reserve Components Common Personnel Data System. The thes
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Licari, Anthony D. "DEVELOPING A MARKOV MODEL FOR FORECASTING END STRENGTH OF SELECTED MARINE CORPS RESERVE (SMCR) OFFICERS." Monterey, California. Naval Postgraduate School, 2013. http://hdl.handle.net/10945/32856.

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This thesis develops Markov models for prior service (PS) and non-prior service (NPS) Selected Marine Corps Reserve (SMCR) officers. Data were collected from the Total Force Data Warehouse (TFDW) for all SMCR officers who served between September 30, 1998 and October 31, 2012. Determining SMCR officer end strength is necessary for reserve manpower planners to balance the force structure, minimize personnel excesses and shortages that impact training and labor costs, and to manage career progression. The PS model validation and analysis show that an aggregate monthly rate and unique monthly tra
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Conley, Gary D. "Examining the Cover and Composition of the Successional Vegetation Mosaic of Pre-SMCRA Mined Landscapes in Southeast Ohio." Ohio University / OhioLINK, 2013. http://rave.ohiolink.edu/etdc/view?acc_num=ohiou1357751096.

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Holsinger, John Frederick. "The Impact of SMCRA on Select Soil Properties in Reclaimed Mine Sites Determined by Geochemical and Hydrological Analyses." The Ohio State University, 2014. http://rave.ohiolink.edu/etdc/view?acc_num=osu1406134738.

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Leveroos, Maura K. "Economic Viability of Woody Bioenergy Cropping for Surface Mine Reclamation." Thesis, Virginia Tech, 2013. http://hdl.handle.net/10919/50950.

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Planting woody biomass for energy production can be used as a mine reclamation procedure to satisfy the Surface Mining Control and Reclamation Act of 1977 (SMCRA) and provide renewable energy for the United States.  This study examines the economic viability of bioenergy production on previously mined lands using multiple hardwood species and treatments.  Five species were planted at two densities; one-half of the trees were fertilized in year two.  Height and diameter of the trees were measured annually for five years; the first three years by cooperating researchers at Virginia Tech, the las
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Prichard, Elizabeth Dulaney. "Sustainable Community Development in Boom and Bust Economies: A Comparative Case Analysis of Institutional Stakeholder Interaction in Four Appalachian Natural Resource Dependent Locales." Diss., Virginia Tech, 2019. http://hdl.handle.net/10919/99286.

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The purpose of this dissertation research is to examine how local context in four natural resource dependent Appalachian settings shape the formal Basic Engagement Plan (BEP). In 2002, the federal Office of Surface Mining, Reclamation and Enforcement (OSMRE) formed a partnership with AmeriCorps VISTA and local environmental improvement organizations to restore Appalachian locales suffering from pre-regulatory environmental damage. To stimulate diverse local institutional engagement, the partnership developed the BEP. It is a formal process of institutional interaction to initiate working relat
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Erhardt, Bruce J. Jr. "Development of a Markov Model for Forecasting Continuation Rates for Enlisted Prior Service and Non-Prior Service Personnel in the Selective Marine Corps Reserve (SMCR)." Thesis, Monterey, California. Naval Postgraduate School, 2012. http://hdl.handle.net/10945/6791.

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The purpose of this thesis is to develop a Markov model to determine the continuation rates for Prior Service and Non-Prior Service enlisted population in the Selected Marine Corps Reserve (SMCR). Determining the end strength for these populations is necessary for reserve manpower planners to balance the force structure to minimize personnel overage or underages that impact training and labor costs, as well as career progression. The results of model validation indicate that models based on annual aggregate monthly transition rates fails the stationarity assumption required of Markov models. T
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Pennington, Michael Sean. "Is the coal industry worth protecting? an examination of the effects of competing advocacy coalitions on implementation of the Surface Mining Control and Reclamation Act (SMCRA) of 1977." Texas A&M University, 2008. http://hdl.handle.net/1969.1/85961.

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Harold Lasswell (1936) defined politics as the exploration of "who gets what, when, and how." As such, one of the central concerns of democratic governance is the role that affected interests play not only in politics, but in the implementation of adopted policies as well. In this dissertation, I use both comparative method case studies, as well as pooled-time series statistical techniques, to examine the effects of political, economic and market forces, and competition between the affected interests on implementation of the Surface Mining Control and Reclamation Act of 1977. The findings of t
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Pollock, Matthew John. "Geomorphic Differences between Unmined and Surface Mined Lands in Southeastern Ohio." The Ohio State University, 2015. http://rave.ohiolink.edu/etdc/view?acc_num=osu1429613137.

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Books on the topic "SMCR8"

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O'Donohue, Daniel J. First-term retention of enlisted Selected Marine Corps (SMCR) Reservists. Naval Postgraduate School, 1988.

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United States. Congress. House. Committee on Interior and Insular Affairs. Subcommittee on Mining and Natural Resources. Implementation of SMCRA: Oversight hearing before the Subcommittee on Mining and Natural Resources of the Committee on Interior and Insular Affairs, House of Representatives, One Hundred Second Congress, second session, on implementation of the Surface Mining Control and Reclamation Act of 1977, hearing held in Washington, DC, February 20, 1992. U.S. G.P.O., 1992.

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Research and technical studies under Title V of SMCRA. The Office, 1992.

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Research and technical studies under Title V of SMCRA. The Office, 1992.

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Gumbel, John D. An evaluation of the Selected Reserve Educational Assistance Program (SREAP) as it relates to the Selected Marine Corps Reserve (SMCR). 1987.

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US GOVERNMENT. Implementation of SMCRA: Oversight hearing before the Subcommittee on Mining and Natural Resources of the Committee on Interior and Insular Affairs, House ... held in Washington, DC, February 20, 1992. For sale by the U.S. G.P.O., Supt. of Docs., Congressional Sales Office, 1992.

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Implementation of SMCRA: Oversight hearing before the Subcommittee on Mining and Natural Resources of the Committee on Interior and Insular Affairs, House of Representatives, One Hundred Second Congress, second session, on implementation of the Surface Mining Control and Reclamation Act of 1977, hearing held in Washington, DC, February 20, 1992. U.S. G.P.O., 1992.

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Book chapters on the topic "SMCR8"

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Allan, Daniel, and Gregory Brandman. "The SMCR Conduct Rules." In Eversheds Sutherland: The Employment Practitioner’s Guide to Financial Institutions: Key Aspects of the Regulatory Framework. Bloomsbury Professional, 2019. http://dx.doi.org/10.5040/9781526504234.chapter-006.

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Henry, Charlotte. "The UK’s Senior Managers, Certification and Conduct Regime." In Brexit and Financial Regulation. Oxford University Press, 2020. http://dx.doi.org/10.1093/law/9780198840794.003.0009.

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Abstract The SMCR regime was first introduced in the UK for banks in March 2016 and is being rolled out to all other financial services firms with effect from 9 December 2019. This chapter focuses primarily on financial services firms that are banks covering the different layers of complexity that a no-deal Brexit will bring to the SMCR requirements. In particular, the chapter covers the difficult position firms may find themselves in when opting into the TPR where the PRA and FCA have adopted different approaches. When entering the TPR, these firms will need to implement a hybrid SMCR regime
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McQuitty, Jake. "FCA Enforcement and the SMCR." In Eversheds Sutherland: The Employment Practitioner’s Guide to Financial Institutions: Key Aspects of the Regulatory Framework. Bloomsbury Professional, 2019. http://dx.doi.org/10.5040/9781526504234.chapter-013.

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Schladweiler, Brenda K. "40 Years of SMCRA: What have we learned in the state of Wyoming." In Land Reclamation in Ecological Fragile Areas. CRC Press, 2017. http://dx.doi.org/10.1201/9781315166582-2.

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Newman, Jane D., and Julia C. van Kessel. "Purification of the Vibrio Quorum-Sensing Transcription Factors LuxR, HapR, and SmcR." In Methods in Molecular Biology. Springer US, 2020. http://dx.doi.org/10.1007/7651_2020_306.

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Conference papers on the topic "SMCR8"

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Prasad, Bandi Vittal, B. Venugopal Rao, K. Narsaiah, G. Narsinga Rao, J. W. Chen, and D. Suresh Babu. "Synthesis and dielectric properties of Fe doped SmCrO[sub 3] semiconductor ceramics." In PROCEEDING OF INTERNATIONAL CONFERENCE ON RECENT TRENDS IN APPLIED PHYSICS AND MATERIAL SCIENCE: RAM 2013. AIP, 2013. http://dx.doi.org/10.1063/1.4810417.

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Song, Wei. "Analysis on Communication Strategies of Food Culture Based on SMCR Model." In 2023 5th International Conference on Applied Machine Learning (ICAML). IEEE, 2023. http://dx.doi.org/10.1109/icaml60083.2023.00040.

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Sugiura, Larissa, Julia da Silva Pimenta, Maria Julia Onça Moreira, et al. "Estudo epidemiológico e molecular de Serratia marcescens, isolados de infecções da corrente sanguínea, durante a pandemia de COVID-19, em um hospital no Sul do Brasil." In Resumos do 56º Congresso Brasileiro de Patologia Clínica/Medicina Laboratorial. Zeppelini Editorial e Comunicação, 2024. https://doi.org/10.5327/1516-3180.142s1.12633.

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Objetivo: Serratia marcescens é um importante agente de infecções relacionadas à assistência à saúde (IRAS) com alta resistência aos antimicrobianos de amplo espectro. Os carbapenêmicos são de primeira escolha na terapia de IRAS, mas o aumento da resistência pela produção de carbapenemases, como Klebsiella pneumoniae carbapenemase (KPC) e New Dehli Metalo-β-lactamase (NDM), torna seu uso limitado. S. marscescens emergiu como um importante patógeno para infecções da corrente sanguínea devido à pandemia por COVID-19 e ao uso de antimicrobianos. O estudo avaliou a disseminação de carbapenemases e
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Li, Ziqi. "Analysis on ‘Role’ Transformation of English Teachers in Chinese Higher Vocational Colleges from Traditional Classroom to Flipped Classroom under SMCR Model." In 7th International Conference on Humanities and Social Science Research (ICHSSR 2021). Atlantis Press, 2021. http://dx.doi.org/10.2991/assehr.k.210519.064.

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