RHBDD1

Entrez Summary: N/A
UniProt Summary: Intramembrane-cleaving serine protease that cleaves single transmembrane or multi-pass membrane proteins in the hydrophobic plane of the membrane, luminal loops and juxtamembrane regions. Involved in regulated intramembrane proteolysis and the subsequent release of functional polypeptides from their membrane anchors. Functional component of endoplasmic reticulum-associated degradation (ERAD) for misfolded membrane proteins. Required for the degradation process of some specific misfolded endoplasmic reticulum (ER) luminal proteins. Participates in the transfer of misfolded proteins from the ER to the cytosol, where they are destroyed by the proteasome in a ubiquitin-dependent manner. Functions in BIK, MPZ, PKD1, PTCRA, RHO, STEAP3 and TRAC processing. Involved in the regulation of exosomal secretion; inhibits the TSAP6-mediated secretion pathway. Involved in the regulation of apoptosis; modulates BIK-mediated apoptotic activity. Also plays a role in the regulation of spermatogenesis; inhibits apoptotic activity in spermatogonia. {ECO:0000269|PubMed:18953687, ECO:0000269|PubMed:22624035}.

Pfam Domains GO Terms

Compound Hit Most Correlated Genes in Chemogenomics Tissues where Essential in the Avana Dataset (DepMap 20Q1)

Global Fraction of Cell Lines Where Essential: 0/739

Tissue	Fraction Of Cell Lines Where Essential
1290807.0	0/1
909776.0	0/1
bile duct	0/28
blood	0/28
bone	0/26
breast	0/33
central nervous system	0/56
cervix	0/4
colorectal	0/17
esophagus	0/13
fibroblast	0/1
gastric	0/16
kidney	0/21
liver	0/20
lung	0/75
lymphocyte	0/16
ovary	0/26
pancreas	0/24
peripheral nervous system	0/16
plasma cell	0/15
prostate	0/1
skin	0/24
soft tissue	0/9
thyroid	0/2
upper aerodigestive	0/22
urinary tract	0/29
uterus	0/5

Expression Distribution

membrane protein proteolysis involved in retrograde protein transport, ER to cytosol
regulation of male germ cell proliferation
regulation of germ cell proliferation
retrograde protein transport, ER to cytosol
endoplasmic reticulum to cytosol transport
membrane protein intracellular domain proteolysis
endoplasmic reticulum quality control compartment
positive regulation of protein processing
positive regulation of protein maturation
protein exit from endoplasmic reticulum
membrane protein proteolysis
regulation of protein processing
regulation of protein maturation
endopeptidase activity
cellular response to UV
ERAD pathway
integral component of endoplasmic reticulum membrane
cellular response to light stimulus
cellular response to unfolded protein
response to UV
cellular response to topologically incorrect protein
spermatid differentiation
regulation of reproductive process
response to unfolded protein
serine-type endopeptidase activity
cellular response to radiation
response to topologically incorrect protein
response to endoplasmic reticulum stress
response to light stimulus
cellular response to environmental stimulus
cellular response to abiotic stimulus
proteasomal protein catabolic process
cellular process involved in reproduction in multicellular organism
positive regulation of proteolysis
post-translational protein modification
response to radiation
spermatogenesis
male gamete generation
proteolysis involved in cellular protein catabolic process
cellular protein catabolic process