ERLIN2
ERLIN2 (ER lipid raft associated 2, also known as Erlin-2, NET32, C8orf2, SPFH2 and SPG18) is an endoplasmic reticulum membrane protein of the band 7/mec-2 family. It forms a complex with ERLIN1 that targets selected proteins for ER-associated degradation. Browse Assay Genie's current range of ERLIN2 research reagents below, or read on for the biology behind the target.
ERLIN2 is a component of the ERLIN1/ERLIN2 complex, which mediates endoplasmic reticulum-associated degradation (ERAD) of inositol 1,4,5-trisphosphate receptors (IP3Rs) such as ITPR1. By driving the removal of activated IP3 receptors, the protein plays a critical role in IP3 signalling. It also promotes sterol-accelerated ERAD of HMGCR, probably involving an AMFR/gp78-containing ubiquitin ligase complex.
Beyond receptor turnover, ERLIN2 is involved in regulating cellular cholesterol homeostasis through the SREBP signalling pathway, and it may promote ER retention of the SCAP-SREBF complex. The ERLIN2 protein is localised to lipid rafts of the endoplasmic reticulum and belongs to the band 7/mec-2 family of SPFH domain-containing, lipid raft-associated proteins.
The ERLIN2 gene was formerly designated chromosome 8 open reading frame 2 (C8orf2) and SPFH domain family, member 2 (SPFH2). Mutations in the gene are a cause of autosomal dominant spastic paraplegia 18 (SPG18), which accounts for the SPG18 alias and the earlier name spastic paraplegia 18. Alternatively spliced transcript variants encoding multiple isoforms have been observed. Mouse and rat both carry an orthologous Erlin2 gene.
ERLIN2 is part of a complex that mediates ERAD of IP3 receptors, making it a useful focus for studies of ER protein quality control.
By mediating degradation of activated IP3 receptors such as ITPR1, ERLIN2 contributes to the regulation of IP3 signalling.
ERLIN2 promotes sterol-accelerated ERAD of HMGCR and regulates the SREBP pathway, linking it to cellular cholesterol control.
Mutations in the ERLIN2 gene cause spastic paraplegia 18, so the protein is relevant to research on this neurological disorder.
ERLIN2 is a component of the ERLIN1/ERLIN2 complex that mediates ER-associated degradation of inositol 1,4,5-trisphosphate receptors. It also promotes sterol-accelerated degradation of HMGCR and helps regulate cholesterol homeostasis through the SREBP signalling pathway, and may promote ER retention of the SCAP-SREBF complex.
ERLIN2 is also called Erlin-2, NET32, C8orf2, SPFH2 and SPG18. Earlier names include chromosome 8 open reading frame 2 and SPFH domain family, member 2. Alternatively spliced transcript variants encoding multiple isoforms have been reported for the gene.
Mutations in ERLIN2 are a cause of spastic paraplegia 18 (SPG18). Orthologous genes named Erlin2 exist in mouse and rat, so rodent models can be used to study the protein. Check the datasheet of any product for its validated reactivity.
The ERLIN2 partner ERLIN1 and related ER-associated degradation targets with products at Assay Genie.
Gene and protein annotations from HGNC, NCBI Gene and UniProt O94905; product specifications from Assay Genie datasheets.