Rhbdd1
entrez
76867
canonical
uniprot
Q8BHC7
canonical
ensembl
ENSMUSG00000026142
resolver
ensp
ENSMUSP00000027322
resolver
ensp
ENSMUSP00000137770
resolver
ensp
ENSMUSP00000234355
resolver
ensp
ENSMUSP00000345848
resolver
enst
ENSMUST00000027322
resolver
enst
ENSMUST00000140020
resolver
genesymbol
Rhbdd1
resolver
genesymbol_syn
Rhbdl4
resolver
uniprot
Q99K13
resolver
uniprot
Q9D5L8
resolver
uniprot_entry
RHBL4_MOUSE
resolver
uniprot_sec
Q99K13
resolver
uniprot_sec
Q9D5L8
resolver
activity_regulation
ACTIVITY REGULATION: Inhibited by aprotinin. {ECO:0000250}.
uniprot
proteins
amino_acid_sequence
MQRRTRGINTGLLLLLSQVFQIGINNIPPVTLATLAVNVWFFLNPWKPLYHSCISVEKCYQQKDWQRLLLSPLHHGDDWHLYFNMVSMLWKGVKLERRLGSRWFAYVIATFSLLTGVVYLLLQFTVAELLNQPDFKRNCAVGFSGVLFALKVLSNHYCPGGFVNILGFPVPNRFACWAELVAIHFCTPGTSFAGHLAGILVGLMYTQGPLKKIMDTCAGIFISHAGPSGQQNHFNNAGPSGYQNHYADGRPVTYDATYRNYDVYTAGLSEEEQLERALRASIWDRGNTRNGPMPYGFRLPPEEMRRQRLHRFDGQ
uniprot
proteins
ec_number
3.4.21.105
uniprot
proteins
function
FUNCTION: 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:19358743, ECO:0000269|PubMed:22795130}.
uniprot
proteins
mass_dalton
35776
uniprot
proteins
mutagenesis
MUTAGEN 144; /note="S->A: Enzyme inactivation. Reduces the cleavage of PTCRA and TRAC. Does not inhibit interaction with PTCRA. Stimulates interaction with ubiquitinated proteins."; /evidence="ECO:0000269|PubMed:22795130"; MUTAGEN 274; /note="L->A: Inhibits interaction with ubiquitin and ubiquitinated proteins; when associated with A-278. Reduces the cleavage of PTCRA; when associated with A-278."; /evidence="ECO:0000269|PubMed:22795130"; MUTAGEN 278; /note="L->A: Inhibits interaction with ubiquitin and ubiquitinated proteins; when associated with A-274. Reduces the cleavage of PTCRA; when associated with A-274."; /evidence="ECO:0000269|PubMed:22795130
uniprot
proteins
protein_family
Peptidase S54 family
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16141072
uniprot
proteins
pubmed
19358743
uniprot
proteins
pubmed
21183079
uniprot
proteins
pubmed
22795130
uniprot
proteins
sequence_length
315
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Endoplasmic reticulum membrane {ECO:0000269|PubMed:22795130}; Multi-pass membrane protein {ECO:0000305}. Mitochondrion membrane {ECO:0000250|UniProtKB:Q8TEB9}; Multi-pass membrane protein {ECO:0000305}.
uniprot
proteins
transmembrane_region
TRANSMEM 22..42; /note="Helical"; /evidence="ECO:0000255"; TRANSMEM 104..124; /note="Helical"; /evidence="ECO:0000255"; TRANSMEM 138..154; /note="Helical"; /evidence="ECO:0000255"; TRANSMEM 181..201; /note="Helical"; /evidence="ECO:0000255
uniprot
proteins