| Gene Symbol |
P4HB
|
| Entrez Gene |
5034
|
| Alt Symbol |
CLCRP1, DSI, ERBA2L, GIT, P4Hbeta, PDI, PDIA1, PHDB, PO4DB, PO4HB, PROHB
|
| Species |
Human
|
| Gene Type |
protein-coding
|
| Description |
prolyl 4-hydroxylase, beta polypeptide
|
| Other Description |
cellular thyroid hormone-binding protein|collagen prolyl 4-hydroxylase beta|glutathione-insulin transhydrogenase|p55|procollagen-proline, 2-oxoglutarate 4-dioxygenase (proline 4-hydroxylase), beta polypeptide|prolyl 4-hydroxylase subunit beta|protein disulfide isomerase family A, member 1|protein disulfide isomerase-associated 1|protein disulfide isomerase/oxidoreductase|protein disulfide-isomerase|protocollagen hydroxylase|thyroid hormone-binding protein p55
|
| Swissprots |
P30037 P32079 Q6LDE5 B2RDQ2 P07237 Q15205
|
| Accessions |
AAB22262 AAC13652 CCQ43001 CDM55388 CDM55569 EAW89690 EAW89691 EAW89692 EAW89693 EAW89694 EAW89695 EAW89696 P07237 AB062434 BAB93497 AK075503 AK095938 BAG53171 AK128783 BAG54732 AK296206 BAG58932 AK297081 BAG59598 AK297967 BAG60277 AK298005 BAG60311 AK300561 BAG62267 AK308600 AK315631 BAG37999 AU131354 BC010859 AAH10859 BC014504 AAH14504 BC018801 BC029617 AAH29617 BC071892 AAH71892 BX353203 CA412209 DB466371 J02783 AAA61169 JF432204 ADZ15421 X05130 CAA28775 X07077 CAA30112 NM_000918 NP_000909
|
| Function |
This multifunctional protein catalyzes the formation, breakage and rearrangement of disulfide bonds. At the cell surface, seems to act as a reductase that cleaves disulfide bonds of proteins attached to the cell. May therefore cause structural modifications of exofacial proteins. Inside the cell, seems to form/rearrange disulfide bonds of nascent proteins. At high concentrations, functions as a chaperone that inhibits aggregation of misfolded proteins. At low concentrations, facilitates aggregation (anti-chaperone activity). May be involved with other chaperones in the structural modification of the TG precursor in hormone biogenesis. Also acts a structural subunit of various enzymes such as prolyl 4-hydroxylase and microsomal triacylglycerol transfer protein MTTP. {ECO:0000269|PubMed:10636893, ECO:0000269|PubMed:12485997}.
|
| Subcellular Location |
Endoplasmic reticulum lumen. Melanosome. Cell membrane {ECO:0000305}; Peripheral membrane protein {ECO:0000305}. Note=Highly abundant. In some cell types, seems to be also secreted or associated with the plasma membrane, where it undergoes constant shedding and replacement from intracellular sources (Probable). Localizes near CD4-enriched regions on lymphoid cell surfaces. Identified by mass spectrometry in melanosome fractions from stage I to stage IV. {ECO:0000305}.
|
| Top Pathways |
Protein processing in endoplasmic reticulum
|