Target intelligence / Profile preview

Translocation protein SEC63 homolog (SEC63)

Target
SEC63
Molecular classification
Other, Endoplasmic reticulum translocon component, Integral membrane protein
01

Overview

Translocation protein SEC63 homolog (SEC63) is an integral membrane protein that is a key component of the endoplasmic reticulum (ER) Sec61 translocon complex, crucial for the translocation of precursor proteins across the ER membrane[1][3][5]. SEC63 mediates both cotranslational and post-translational import of proteins into the ER and plays an auxiliary role in recognizing substrates with short, hydrophobic signal peptides[1][3][5]. It works in concert with SEC62 and HSPA5/BiP to facilitate the entry of small presecretory proteins into the SEC61 channel, enabling their passage into the ER lumen[1][5]. SEC63 is also implicated in the backward (retrograde) transport of ER proteins destined for ubiquitin-proteasome degradation[3][5]. Mutations in the SEC63 gene cause autosomal dominant polycystic liver disease and can contribute to concurrent polycystic kidney disease[2][5]. There is no current evidence that SEC63 serves as a direct therapeutic target or that any approved drugs interact specifically with this protein.

Other names
DNAJC23ERdj2PCLD2PRO2507SEC63LSEC63 protein translocation regulatorDnaJ homolog subfamily C member 23
02

Biological functions

Protein translocationProtein traffickingProtein quality controlEndoplasmic reticulum-associated degradation (ERAD)Cotranslational and post-translational protein transport
03

Disease associations

Polycystic liver diseasePolycystic kidney diseaseGenetic disorder
04

Biomarkers

SEC63 mutation (as a genetic biomarker for polycystic liver disease)

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