SEC63

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]

Full Name

SEC63 homolog (S. cerevisiae)

Function

Mediates cotranslational and post-translational transport of certain precursor polypeptides across endoplasmic reticulum (ER) (PubMed:22375059, PubMed:29719251).
Proposed to play an auxiliary role in recognition of precursors with short and apolar signal peptides. May cooperate with SEC62 and HSPA5/BiP to facilitate targeting of small presecretory proteins into the SEC61 channel-forming translocon complex, triggering channel opening for polypeptide translocation to the ER lumen (PubMed:29719251).
Required for efficient PKD1/Polycystin-1 biogenesis and trafficking to the plasma membrane of the primary cilia (By similarity).

Biological Process

Biological Process liver developmentIEA:Ensembl
Biological Process multicellular organism agingIEA:Ensembl
Biological Process nitrogen compound metabolic processIEA:Ensembl
Biological Process post-translational protein targeting to endoplasmic reticulum membraneManual Assertion Based On ExperimentIMP:MGI
Biological Process post-translational protein targeting to membrane, translocationManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process protein targeting to membraneManual Assertion Based On ExperimentTAS:ProtInc
Biological Process SRP-dependent cotranslational protein targeting to membraneManual Assertion Based On ExperimentIMP:MGI

Cellular Location

Endoplasmic reticulum membrane

Involvement in disease

Polycystic liver disease 2 with or without kidney cysts (PCLD2):
An autosomal dominant hepatobiliary disease characterized by overgrowth of biliary epithelium and supportive connective tissue, resulting in multiple liver cysts. A subset of patients may develop kidney cysts that usually do not result in clinically significant renal disease.

Topology

Lumenal: 1-14
Helical: 15-35
Cytoplasmic: 36-69
Helical: 70-90
Lumenal: 91-188
Helical: 189-209
Cytoplasmic: 210-760