Protein accumulation and neurodegeneration in the woozy mutant mouse is caused by disruption of SIL1, a cochaperone of BiP
about
A SEL1L mutation links a canine progressive early-onset cerebellar ataxia to the endoplasmic reticulum-associated protein degradation (ERAD) machineryMutation in archain 1, a subunit of COPI coatomer complex, causes diluted coat color and Purkinje cell degenerationEssential role of the unfolded protein response regulator GRP78/BiP in protection from neuronal apoptosisThe nucleotide exchange factors of Hsp70 molecular chaperonesProtein disulfide isomerases in neurodegeneration: from disease mechanisms to biomedical applicationsBiP and its nucleotide exchange factors Grp170 and Sil1: mechanisms of action and biological functionsOrchestration of secretory protein folding by ER chaperonesEssential role of the nuclear isoform of RBFOX1, a candidate gene for autism spectrum disorders, in the brain development.Functional Role of the Disulfide Isomerase ERp57 in Axonal RegenerationLate-onset of spinal neurodegeneration in knock-in mice expressing a mutant BiPA deficiency of ceramide biosynthesis causes cerebellar purkinje cell neurodegeneration and lipofuscin accumulationStructural analysis of the Sil1-Bip complex reveals the mechanism for Sil1 to function as a nucleotide-exchange factorMolecular chaperones of the Hsp110 family act as nucleotide exchange factors of Hsp70s.The gene disrupted in Marinesco-Sjögren syndrome encodes SIL1, an HSPA5 cochaperoneER chaperones in mammalian development and human diseasesElevation of 20-carbon long chain bases due to a mutation in serine palmitoyltransferase small subunit b results in neurodegenerationCharacterization of Zebrafish Models of Marinesco-Sjögren SyndromeEditing-defective tRNA synthetase causes protein misfolding and neurodegenerationMutation of the BiP/GRP78 gene causes axon outgrowth and fasciculation defects in the thalamocortical connections of the mammalian forebrainReduced translocation of nascent prion protein during ER stress contributes to neurodegeneration.Hereditary spastic paraplegia-associated mutations in the NIPA1 gene and its Caenorhabditis elegans homolog trigger neural degeneration in vitro and in vivo through a gain-of-function mechanismA nationwide survey on Marinesco-Sjögren syndrome in Japan.Regulation of basal cellular physiology by the homeostatic unfolded protein response2-Deoxy-D-glucose regulates dedifferentiation through beta-catenin pathway in rabbit articular chondrocytesMutation of a U2 snRNA gene causes global disruption of alternative splicing and neurodegenerationRNA function. Ribosome stalling induced by mutation of a CNS-specific tRNA causes neurodegeneration.Discovery Genetics - The History and Future of Spontaneous Mutation Research.A mouse model of generalized non-Herlitz junctional epidermolysis bullosa.Cellular commitment in the developing cerebellumOverproduction and biophysical characterization of human HSP70 proteins.Hepatocyte-specific ablation of Foxa2 alters bile acid homeostasis and results in endoplasmic reticulum stress.Comparison of the enzymatic and functional properties of three cytosolic carboxypeptidase family membersLoss of the tuberous sclerosis complex protein tuberin causes Purkinje cell degenerationEndoplasmic reticulum stress and neurodegeneration in rats neonatally infected with borna disease virus.Sil1, a nucleotide exchange factor for BiP, is not required for antibody assembly or secretionGRP78/BiP is required for cell proliferation and protecting the inner cell mass from apoptosis during early mouse embryonic development.Antigen retrieval to improve the immunocytochemistry detection of sigma-1 receptors and ER chaperones.C-terminal mutations destabilize SIL1/BAP and can cause Marinesco-Sjögren syndrome.Activation of ER Stress and Autophagy Induced by TDP-43 A315T as Pathogenic Mechanism and the Corresponding Histological Changes in Skin as Potential Biomarker for ALS with the MutationThe impact of the unfolded protein response on human disease
P2860
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P2860
Protein accumulation and neurodegeneration in the woozy mutant mouse is caused by disruption of SIL1, a cochaperone of BiP
description
2005 nî lūn-bûn
@nan
2005 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
2005 թվականի սեպտեմբերին հրատարակված գիտական հոդված
@hy
2005年の論文
@ja
2005年論文
@yue
2005年論文
@zh-hant
2005年論文
@zh-hk
2005年論文
@zh-mo
2005年論文
@zh-tw
2005年论文
@wuu
name
Protein accumulation and neuro ...... of SIL1, a cochaperone of BiP
@ast
Protein accumulation and neuro ...... of SIL1, a cochaperone of BiP
@en
Protein accumulation and neuro ...... of SIL1, a cochaperone of BiP
@nl
type
label
Protein accumulation and neuro ...... of SIL1, a cochaperone of BiP
@ast
Protein accumulation and neuro ...... of SIL1, a cochaperone of BiP
@en
Protein accumulation and neuro ...... of SIL1, a cochaperone of BiP
@nl
prefLabel
Protein accumulation and neuro ...... of SIL1, a cochaperone of BiP
@ast
Protein accumulation and neuro ...... of SIL1, a cochaperone of BiP
@en
Protein accumulation and neuro ...... of SIL1, a cochaperone of BiP
@nl
P2093
P2860
P3181
P356
P1433
P1476
Protein accumulation and neuro ...... of SIL1, a cochaperone of BiP
@en
P2093
Belinda S Harris
Chantal Longo-Guess
Jeong-Woong Lee
Susan L Ackerman
P2860
P2888
P3181
P356
10.1038/NG1620
P407
P50
P577
2005-09-01T00:00:00Z