SEC63


Description

The SEC63 (SEC63 homolog, protein translocation regulator) is a protein-coding gene located on chromosome 6.

SEC63 is a protein involved in protein translocation across the ER membrane. It is part of the Sec61 complex, which is essential for protein transport into the ER. SEC63 may play a role in post-translational protein translocation, as well as backward transport of ER proteins that are degraded. Mutations in the SEC63 gene have been linked to autosomal dominant polycystic liver disease.

SEC63 facilitates the movement of certain precursor proteins across the endoplasmic reticulum (ER) membrane, both during and after protein synthesis (co-translational and post-translational transport). It helps recognize precursor proteins with short and hydrophobic signal sequences. SEC63 may work together with SEC62 and HSPA5/BiP to guide small presecretory proteins into the SEC61 translocon complex, which then opens to allow the protein to pass into the ER lumen. SEC63 is essential for the proper formation and transport of PKD1/Polycystin-1 to the primary cilia.

SEC63 is also known as DNAJC23, ERdj2, PCLD2, PRO2507, SEC63L.

Associated Diseases


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