Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
endoplasmic reticulum | - |
Homo sapiens | 5783 | - |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | Q8TEB9 | - |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
HEK-293T cell | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
alpha chain of pre-T cell receptor + H2O | constitutively active receptor variant required for T cell development. cleavage contributes to ER-associated degradation, cleavage productsare translocated and degraded by the proteasome | Homo sapiens | ? | - |
? | |
additional information | positively charged transmembrane residues promote isoform RHBDL4-catalyzed cleavage | Homo sapiens | ? | - |
? | |
myelin protein zero mutant L170R + H2O | mutant form is unstable and efficiently cleaved by isoform RHBDL4. Wild-type myelin protein zero is not a substrate | Homo sapiens | ? | - |
? | |
opsin mutant bearing TCRalpha degron motif + H2O | opsin-degron mutant is degraded by isoform RHBDL4, whereas the wild-type protein is stable | Homo sapiens | ? | - |
? | |
polycystin-1 + H2O | 11-TM spanning membrane protein. Isoform RHBDL4 cleaves several truncated versions of polycystin-1 at luminal loops or juxtamembrane transmembrane regions. Wild-type olycystin-1 is not a substrate | Homo sapiens | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
Rhbdd1 | - |
Homo sapiens |
RHBDL4 | - |
Homo sapiens |
rhomboid-related protein 4 | - |
Homo sapiens |
Organism | Comment | Expression |
---|---|---|
Homo sapiens | treatment with tunicamycin leads to increase level in enzyme protein | up |
General Information | Comment | Organism |
---|---|---|
physiological function | isoform RHBDL4 is a ubiquitin-dependent ER-resident intramembrane protease that is upregulated upon ER stress. RHBDL4 cleaves single-spanning and polytopic membrane proteins with unstable transmembrane helices, leading to their degradation by the canonical ER-associated degradation machinery and binds ubiquitin in Its conserved C-terminal domain. RHBDL4 specifically binds the AAA+-ATPase p97 | Homo sapiens |