SEL1L

The protein encoded by this gene is part of a protein complex required for the retrotranslocation or dislocation of misfolded proteins from the endoplasmic reticulum lumen to the cytosol, where they are degraded by the proteasome in a ubiquitin-dependent manner. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.

Full Name

SEL1L ERAD E3 Ligase Adaptor Subunit

Function

Plays a role in the endoplasmic reticulum quality control (ERQC) system also called ER-associated degradation (ERAD) involved in ubiquitin-dependent degradation of misfolded endoplasmic reticulum proteins (PubMed:16186509, PubMed:29997207).
Enhances SYVN1 stability. Plays a role in LPL maturation and secretion. Required for normal differentiation of the pancreas epithelium, and for normal exocrine function and survival of pancreatic cells. May play a role in Notch signaling.

Biological Process

Biological Process ERAD pathwayManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process Notch signaling pathwayIEA:UniProtKB-KW
Biological Process protein secretionISS:UniProtKB
Biological Process protein stabilizationManual Assertion Based On ExperimentTAS:ParkinsonsUK-UCL
Biological Process retrograde protein transport, ER to cytosolManual Assertion Based On ExperimentIMP:ParkinsonsUK-UCL
Biological Process triglyceride metabolic processISS:UniProtKB
Biological Process ubiquitin-dependent ERAD pathwayManual Assertion Based On ExperimentTAS:ParkinsonsUK-UCL

Cellular Location

Endoplasmic reticulum membrane

Topology

Lumenal: 22-738
Helical: 739-759
Cytoplasmic: 760-794

PTM

N-glycosylated.