P4HB


Summary: This gene encodes the beta subunit of prolyl 4-hydroxylase, a highly abundant multifunctional enzyme that belongs to the protein disulfide isomerase family. When present as a tetramer consisting of two alpha and two beta subunits, this enzyme is involved in hydroxylation of prolyl residues in preprocollagen. This enzyme is also a disulfide isomerase containing two thioredoxin domains that catalyze the formation, breakage and rearrangement of disulfide bonds. Other known functions include its ability to act as a chaperone that inhibits aggregation of misfolded proteins in a concentration-dependent manner, its ability to bind thyroid hormone, its role in both the influx and efflux of S-nitrosothiol-bound nitric oxide, and its function as a subunit of the microsomal triglyceride transfer protein complex. [provided by RefSeq, Jul 2008].

NameOMIM IDEnsembl IDHGNC IDPHARMGKB IDMap Location
prolyl 4-hydroxylase subunit betaMIM:176790Ensembl:ENSG00000185624HGNC:HGNC:8548PA3287617q25.3

GO terms in P4HB


Term TypeEvidence TypeGO Term IDGO Des.
BPIBAGO:0006457protein folding
BPIDAGO:0018401peptidyl-proline hydroxylation to 4-hydroxy-L-proline
BPTASGO:0034378chylomicron assembly
BPTASGO:0034379very-low-density lipoprotein particle assembly
BPIBAGO:0034976response to endoplasmic reticulum stress
BPIMPGO:0034976response to endoplasmic reticulum stress
BPTASGO:0035722interleukin-12-mediated signaling pathway
BPTASGO:0038155interleukin-23-mediated signaling pathway
BPTASGO:0043687post-translational protein modification
BPTASGO:0044267cellular protein metabolic process
BPIEAGO:0045454cell redox homeostasis
BPIMPGO:0046598positive regulation of viral entry into host cell
BPIEAGO:0055114oxidation-reduction process
BPIMPGO:0071456cellular response to hypoxia
BPIBAGO:1902175regulation of oxidative stress-induced intrinsic apoptotic signaling pathway
BPIMPGO:1902175regulation of oxidative stress-induced intrinsic apoptotic signaling pathway
CCNASGO:0005576extracellular region
CCIBAGO:0005783endoplasmic reticulum
CCIDAGO:0005783endoplasmic reticulum
CCTASGO:0005788endoplasmic reticulum lumen
CCIDAGO:0005793endoplasmic reticulum-Golgi intermediate compartment
CCHDAGO:0005925focal adhesion
CCIDAGO:0009897external side of plasma membrane
CCIDAGO:0016222procollagen-proline 4-dioxygenase complex
CCIEAGO:0034663endoplasmic reticulum chaperone complex
CCIEAGO:0042470melanosome
CCHDAGO:0070062extracellular exosome
MFHDAGO:0003723RNA binding
MFEXPGO:0003756protein disulfide isomerase activity
MFIBAGO:0003756protein disulfide isomerase activity
MFIDAGO:0003756protein disulfide isomerase activity
MFIDAGO:0004656procollagen-proline 4-dioxygenase activity
MFIPIGO:0005178integrin binding
MFIPIGO:0005515protein binding
MFIDAGO:0015037peptide disulfide oxidoreductase activity
MFIEAGO:0019899enzyme binding
MFIBAGO:0031545peptidyl-proline 4-dioxygenase activity
MFIDAGO:0046982protein heterodimerization activity

Gene expression in normal tissue: P4HB

Gene-model tissue-cancer distribution: Bubble Plot

Gene-drug pathway distribution

Pathways in P4HB


DatabasePathway IDPathway Des.
kegghsa04141Protein processing in endoplasmic reticulum - Homo sapiens (human)
reactomeR-HSA-1280215Cytokine Signaling in Immune system
reactomeR-HSA-1474244Extracellular matrix organization
reactomeR-HSA-1474290Collagen formation
reactomeR-HSA-162582Signal Transduction
reactomeR-HSA-1650814Collagen biosynthesis and modifying enzymes
reactomeR-HSA-168256Immune System
reactomeR-HSA-174824Plasma lipoprotein assembly, remodeling, and clearance
reactomeR-HSA-2262752Cellular responses to stress
reactomeR-HSA-3299685Detoxification of Reactive Oxygen Species
reactomeR-HSA-381426Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)
reactomeR-HSA-382551Transport of small molecules
reactomeR-HSA-392499Metabolism of proteins
reactomeR-HSA-447115Interleukin-12 family signaling
reactomeR-HSA-449147Signaling by Interleukins
reactomeR-HSA-5358346Hedgehog ligand biogenesis
reactomeR-HSA-5358351Signaling by Hedgehog
reactomeR-HSA-597592Post-translational protein modification
reactomeR-HSA-8866423VLDL assembly
reactomeR-HSA-8953897Cellular responses to external stimuli
reactomeR-HSA-8957275Post-translational protein phosphorylation
reactomeR-HSA-8963888Chylomicron assembly
reactomeR-HSA-8963898Plasma lipoprotein assembly
reactomeR-HSA-9020591Interleukin-12 signaling
reactomeR-HSA-9020933Interleukin-23 signaling
biocartahifpathwayhypoxia-inducible factor in the cardivascular system

Gene-Drug: Aster Plot


Drug IDDrug NameModel Num.
iGMDRD255SB2250025
iGMDRD138PX 125
iGMDRD52Rotenone2
iGMDRD870BRD636102
iGMDRD782DC-45-A22
iGMDRD171Pemetrexed2
iGMDRD562Navitoclax2
iGMDRD100Zebularine2
iGMDRD188Piperlongumine8
iGMDRD74Idarubicin2
iGMDRD356PNU-746543
iGMDRD60Quinoclamine2
iGMDRD64Parbendazole3
iGMDRD420Leucascandrolide A3
iGMDRD494Neopeltolide2
iGMDRD781Sirolimus3
iGMDRD144NSC953976
iGMDRD152179324-69-73
iGMDRD446LY 21832403
iGMDRD297Austocystin D3
iGMDRD280CYTOCHALASIN B2
iGMDRD394BX-7952
iGMDRD466Chaetocin4
iGMDRD103SN-388
iGMDRD251ML1623
iGMDRD121GMX17783
iGMDRD267Oligomycin A3
iGMDRD105Triptolide4
iGMDRD329Merck605
iGMDRD414MST-3126
iGMDRD286Nsc 63283910
iGMDRD872BRD71373
iGMDRD353PD03259012
iGMDRD886Compound 1541A2
iGMDRD263AACOCF33
iGMDRD246NPC265
iGMDRD84Lovastatin acid2
iGMDRD330BRD41324
iGMDRD116CD4373
iGMDRD132(-)-gallocatechin-3-O-gallate4
iGMDRD945ML2143
iGMDRD901ELCPK1
iGMDRD123Isoevodiamine3

Gene in drug-gene network: Network Plot

Gene-drug targets distribution

Gene Structure: PDB

Models in P4HB

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