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SEC63 Sec63p

SEC63, Sec63p
The Sec61 complex is the central component of the protein translocation apparatus of the endoplasmic reticulum (ER) membrane. The protein encoded by this gene and SEC62 protein are found to be associated with ribosome-free SEC61 complex. It is speculated that Sec61-Sec62-Sec63 may perform post-translational protein translocation into the ER. The Sec61-Sec62-Sec63 complex might also perform the backward transport of ER proteins that are subject to the ubiquitin-proteasome-dependent degradation pathway. The encoded protein is an integral membrane protein located in the rough ER. [provided by RefSeq, Jul 2008] (from NCBI)
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Top mentioned proteins: Sec61, GRP78, CAN, HSP70, V1a
Papers on SEC63
Severe Polycystic Liver Disease Is Not Caused by Large Deletions of the PRKCSH Gene.
Drenth et al., Nijmegen, Netherlands. In J Clin Lab Anal, Oct 2015
Routine molecular testing using Sanger sequencing identifies pathogenic variants in the PRKCSH (15%) and SEC63 (where SEC63 is Saccharomyces cerevisiae homolog 63 (MIM*608648); 6%) genes, but about approximately 80% of patients meeting the clinical ADPLD criteria carry no PRKCSH or SEC63 mutation.
Comparative Haploid Genetic Screens Reveal Divergent Pathways in the Biogenesis and Trafficking of Glycophosphatidylinositol-Anchored Proteins.
Shen et al., Boulder, United States. In Cell Rep, Jul 2015
SEC62 and SEC63, which encode components of the ER-targeting machinery, were selectively recovered in the PrP screen, indicating that they do not constitute a universal route for the biogenesis of mammalian GPI-APs.
Identification of Biomarkers Associated With Alzheimer's Disease by Bioinformatics Analysis.
Li et al., Jinan, China. In Am J Alzheimers Dis Other Demen, Jul 2015
The target genes SEC22 vesicle trafficking protein homolog B (SEC22B) and SEC63 homolog (SEC63) regulated by miRNA-206, RAB10, member RAS oncogene family (RAB10) regulated by miRNA-655, and fms-related tyrosine kinase 1 (FLT1) regulated by miRNA-30e-3p and miRNA-369-3p were involved in the biological processes of protein transport and regulation of cell motion.
Sec63 and Xbp1 regulate IRE1α activity and polycystic disease severity.
Lee et al., In J Clin Invest, May 2015
The HSP40 cochaperone SEC63 is associated with the SEC61 translocon complex in the ER.
Phosphorylation of the TATA-binding protein activates the spliced leader silencing pathway in Trypanosoma brucei.
Michaeli et al., Ramat Gan, Israel. In Sci Signal, 2014
We found that during ER stress induced by SEC63 silencing or low pH, the serine-threonine kinase PK3 translocated from the ER to the nucleus, where it phosphorylated the TATA-binding protein TRF4, leading to the dissociation of the transcription preinitiation complex from the promoter of the SL RNA encoding gene.
Polycystic liver disease: ductal plate malformation and the primary cilium.
Drenth et al., Nijmegen, Netherlands. In Trends Mol Med, 2014
Polycystic livers are found in autosomal dominant polycystic kidney disease (ADPKD), caused by polycystic kidney disease (PKD)1 and PKD2 mutations in virtually all cases, and in isolated polycystic liver disease (PCLD), where 20% of cases are caused by mutations in Protein kinase C substrate 80K-H (PRKCSH) or SEC63.
Polycystic liver disease: an overview of pathogenesis, clinical manifestations and management.
Drenth et al., Nijmegen, Netherlands. In Orphanet J Rare Dis, 2013
Screening mutations of the genes causing PCLD (PRKCSH and SEC63) or ADPKD (PKD1 and PKD2) confirm the clinical diagnosis.
A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formation.
Somlo et al., New Haven, United States. In Nat Genet, 2011
Autosomal dominant polycystic liver disease results from mutations in PRKCSH or SEC63.
An interaction between human Sec63 and nucleoredoxin may provide the missing link between the SEC63 gene and polycystic liver disease.
Zimmermann et al., Homburg, Germany. In Febs Lett, 2011
identified nucleoredoxin as an interaction partner of Sec63; characterized this interaction; Sec63 is linked to the Wnt signaling pathways and this interaction may be the reason why mutations in SEC63 can lead to polycystic liver disease
Secondary and tertiary structure modeling reveals effects of novel mutations in polycystic liver disease genes PRKCSH and SEC63.
Drenth et al., Nijmegen, Netherlands. In Clin Genet, 2010
identified a total of 26 novel mutations in PRKCSH (n = 14) and SEC63 (n = 12), including four splice site mutations, eight insertions/ deletions, six non-sense mutations, and eight missense mutations
Congenital disorders of glycosylation in hepatology: the example of polycystic liver disease.
Drenth et al., Nijmegen, Netherlands. In J Hepatol, 2010
Disease causing mutations in PRKCSH or SEC63 are found in approximately 25% of the PCLD patients.
The uterine expression of SEC63 gene is up-regulated at implantation sites in association with the decidualization during the early pregnancy in mice.
Wang et al., Xiamen, China. In Reprod Biol Endocrinol, 2008
SEC63 gene expression is up-regulated and predominantly localized in mouse decidual cells during days 5-8 of pregnancy.
Cysts of PRKCSH mutated polycystic liver disease patients lack hepatocystin but express Sec63p.
Drenth et al., Nijmegen, Netherlands. In Histochem Cell Biol, 2008
Sec63p expression was observed in all cyst epithelia regardless of mutational state. And, Cystogenesis in SEC63-associated PCLD occurs via a different mechanism.
Enhanced secretion of heterologous proteins in Pichia pastoris following overexpression of Saccharomyces cerevisiae chaperone proteins.
Liu et al., Beijing, China. In Biotechnol Prog, 2006
The introduction of Sec63 improves secretory proteins in Pichia pastoris secretion 4-7 times.
Management of polycystic liver disease.
Taylor et al., Denver, United States. In Curr Gastroenterol Rep, 2005
Mutations in two distinct genes predispose to renal and liver cysts (PKD1 and PKD2), and mutations in two different genes yield isolated liver cysts (PRKCSH and SEC63).
Polycystic liver and kidney diseases.
Höckerstedt et al., Helsinki, Finland. In Ann Med, 2004
Defects in genes, which code the hepatocystin and SEC63 proteins, have just recently been found to cause PCLD.
Mutations in SEC63 cause autosomal dominant polycystic liver disease.
Somlo et al., New Haven, United States. In Nat Genet, 2004
Mutations in SEC63 cause autosomal dominant polycystic liver disease, suggesting a role for cotranslational protein-processing pathways in maintaining epithelial luminal structure and implicating noncilial ER proteins in human polycystic disease.
BiP acts as a molecular ratchet during posttranslational transport of prepro-alpha factor across the ER membrane.
Rapoport et al., Boston, United States. In Cell, 1999
Multiple BiP molecules associate with each translocation substrate following interaction with the J domain of the Sec63p component of the Sec complex.
Mutant analysis links the translocon and BiP to retrograde protein transport for ER degradation.
Wolf et al., Stuttgart, Germany. In Nature, 1997
The endoplasmic reticulum lumenal chaperone BiP (Kar2p) and Sec63p, which are also subunits of the import machinery, are involved in export of CPY* to the cytosol.
Binding of secretory precursor polypeptides to a translocon subcomplex is regulated by BiP.
Schekman et al., Berkeley, United States. In Cell, 1997
This release reaction, which is specific to BiP and requires interaction between BiP and the DnaJ homolog Sec63p, defines a role for BiP and Sec63p early in the ER translocation process.
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