| IED ID | IndEnz0002009112 |
| Enzyme Type ID | protease009112 |
| Protein Name |
Rhomboid-related protein 4 RRP4 EC 3.4.21.105 Rhomboid domain-containing protein 1 Rhomboid-like protein 4 |
| Gene Name | RHBDD1 RHBDL4 HSD-50 HSD50 |
| Organism | Homo sapiens (Human) |
| Taxonomic Lineage | cellular organisms Eukaryota Opisthokonta Metazoa Eumetazoa Bilateria Deuterostomia Chordata Craniata Vertebrata Gnathostomata (jawed vertebrates) Teleostomi Euteleostomi Sarcopterygii Dipnotetrapodomorpha Tetrapoda Amniota Mammalia Theria Eutheria Boreoeutheria Euarchontoglires Primates Haplorrhini Simiiformes Catarrhini Hominoidea (apes) Hominidae (great apes) Homininae Homo Homo sapiens (Human) |
| Enzyme Sequence | MQRRSRGINTGLILLLSQIFHVGINNIPPVTLATLALNIWFFLNPQKPLYSSCLSVEKCYQQKDWQRLLLSPLHHADDWHLYFNMASMLWKGINLERRLGSRWFAYVITAFSVLTGVVYLLLQFAVAEFMDEPDFKRSCAVGFSGVLFALKVLNNHYCPGGFVNILGFPVPNRFACWVELVAIHLFSPGTSFAGHLAGILVGLMYTQGPLKKIMEACAGGFSSSVGYPGRQYYFNSSGSSGYQDYYPHGRPDHYEEAPRNYDTYTAGLSEEEQLERALQASLWDRGNTRNSPPPYGFHLSPEEMRRQRLHRFDSQ |
| Enzyme Length | 315 |
| Uniprot Accession Number | Q8TEB9 |
| Absorption | |
| Active Site | ACT_SITE 144; /note=Nucleophile; /evidence=ECO:0000250; ACT_SITE 195; /evidence=ECO:0000250 |
| Activity Regulation | ACTIVITY REGULATION: Inhibited by aprotinin. {ECO:0000269|PubMed:18953687}. |
| Binding Site | |
| Calcium Binding | |
| catalytic Activity | CATALYTIC ACTIVITY: Reaction=Cleaves type-1 transmembrane domains using a catalytic dyad composed of serine and histidine that are contributed by different transmembrane domains.; EC=3.4.21.105; |
| DNA Binding | |
| EC Number | 3.4.21.105 |
| Enzyme 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:18953687, ECO:0000269|PubMed:22624035}. |
| Temperature Dependency | |
| PH Dependency | |
| Pathway | |
| nucleotide Binding | |
| Features | Active site (2); Alternative sequence (2); Chain (1); Compositional bias (1); Helix (1); Mutagenesis (2); Natural variant (1); Region (3); Sequence conflict (1); Topological domain (5); Transmembrane (4) |
| Keywords | 3D-structure;Alternative splicing;Apoptosis;Differentiation;Endoplasmic reticulum;Hydrolase;Membrane;Mitochondrion;Protease;Reference proteome;Serine protease;Spermatogenesis;Transmembrane;Transmembrane helix |
| Interact With | Q99675; Q96DZ9-2; Q96BA8; P04921; Q7L5N7; Q15738; Q96CS7; Q59EV6; Q99942; O75396; Q8N2U9; Q69YG0; Q8N661 |
| Induction | INDUCTION: Up-regulated by endoplasmic reticulum stress agents that induce the unfolded protein response (UPR). {ECO:0000269|PubMed:22795130}. |
| Subcellular Location | SUBCELLULAR LOCATION: Endoplasmic reticulum membrane {ECO:0000269|PubMed:22795130}; Multi-pass membrane protein {ECO:0000255}. Mitochondrion membrane {ECO:0000269|PubMed:18953687}; Multi-pass membrane protein {ECO:0000255}. |
| Modified Residue | |
| Post Translational Modification | |
| Signal Peptide | |
| Structure 3D | X-ray crystallography (1) |
| Cross Reference PDB | 5EPP; |
| Mapped Pubmed ID | 17903307; 23534782; 23669365; 23883433; 25260751; 27407164; 27563067; 28445956; 29426364; 30143535; 30286765; 31177093; 31243644; 31693935; 32528541; |
| Motif | |
| Gene Encoded By | |
| Mass | 35,823 |
| Kinetics | |
| Metal Binding | |
| Rhea ID | |
| Cross Reference Brenda | 3.4.21.105; |