Any feedback?
Please rate this page
(literature.php)
(0/150)

BRENDA support

Literature summary for 3.4.21.105 extracted from

  • Fleig, L.; Bergbold, N.; Sahasrabudhe, P.; Geiger, B.; Kaltak, L.; Lemberg, M.K.
    Ubiquitin-dependent intramembrane rhomboid protease promotes ERAD of membrane proteins (2012), Mol. Cell, 47, 558-569.
    View publication on PubMed

Localization

Localization Comment Organism GeneOntology No. Textmining
endoplasmic reticulum
-
Homo sapiens 5783
-

Organism

Organism UniProt Comment Textmining
Homo sapiens Q8TEB9
-
-

Source Tissue

Source Tissue Comment Organism Textmining
HEK-293T cell
-
Homo sapiens
-

Substrates and Products (Substrate)

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

Synonyms Comment Organism
Rhbdd1
-
Homo sapiens
RHBDL4
-
Homo sapiens
rhomboid-related protein 4
-
Homo sapiens

Expression

Organism Comment Expression
Homo sapiens treatment with tunicamycin leads to increase level in enzyme protein up

General Information

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