SYVN1
Description
The SYVN1 (synoviolin 1) is a protein-coding gene located on chromosome 11.
SYVN1 is a gene that encodes an enzyme called synoviolin, which is an E3 ubiquitin-protein ligase. This enzyme is involved in the endoplasmic reticulum (ER)-associated degradation process, where it removes unfolded proteins that have accumulated during ER stress. SYVN1 facilitates the retrograde transport of these proteins from the ER to the cytosol, where they are then degraded by the ubiquitin-proteasome system. Two different isoforms of SYVN1 are produced from different transcript variants.
SYVN1 is an E3 ubiquitin ligase that specifically accepts ubiquitin from the endoplasmic reticulum-associated UBC7 E2 ligase and transfers it to substrates, promoting their degradation. This protein plays a crucial role in the endoplasmic reticulum quality control (ERQC) system, also known as ER-associated degradation (ERAD), by degrading misfolded endoplasmic reticulum proteins through ubiquitination. SYVN1 also promotes the degradation of normal but naturally short-lived proteins, such as SGK. It protects cells from ER stress-induced apoptosis and neurons from apoptosis triggered by polyglutamine-expanded huntingtin (HTT) or unfolded GPR37 by promoting their degradation. Additionally, SYVN1 sequesters p53/TP53 in the cytoplasm and promotes its degradation, thus negatively regulating its biological function in transcription, cell cycle regulation, and apoptosis. It also mediates the ubiquitination and subsequent degradation of cytoplasmic NFE2L1. During early B cell development, SYVN1 is required for degradation of the pre-B cell receptor (pre-BCR) complex, supporting further differentiation into mature B cells.
SYVN1 is also known as DER3, HRD1.
