Biology:SEC63

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A representation of the 3D structure of the protein myoglobin showing turquoise α-helices.
Generic protein structure example

Translocation protein SEC63 homolog is a protein that in humans is encoded by the SEC63 gene.[1][2][3]

Function

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.[3]

Clinical significance

Mutations of this gene have been linked with autosomal dominant polycystic liver disease.[4]

References

  1. "Human Sec63 endoplasmic reticulum membrane protein, map position 6q21". Chromosome Res 7 (1): 77. Jun 1999. doi:10.1023/A:1009283530544. PMID 10219736. 
  2. "Molecular characterization of a novel mammalian DnaJ-like Sec63p homolog". Biol Chem 380 (9): 1133–8. Nov 1999. doi:10.1515/BC.1999.142. PMID 10543453. 
  3. 3.0 3.1 "Entrez Gene: SEC63 SEC63 homolog (S. cerevisiae)". https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=11231. 
  4. "Mutations in SEC63 cause autosomal dominant polycystic liver disease". Nat. Genet. 36 (6): 575–7. June 2004. doi:10.1038/ng1357. PMID 15133510. 

Further reading