| 3244 | P4hb | prolyl 4-hydroxylase subunit beta | ENCODES a protein that exhibits enzyme binding; protein disulfide isomerase activity; protein-containing complex binding; INVOLVED IN cellular response to hypoxia (ortholog); cellular response to interleukin-7 (ortholog); insulin processing (ortholog); PARTICIPATES IN Endoplasmic Reticulum-associate d degradation pathway; ASSOCIATED WITH BCARD syndrome (ortholog); Chemical and Drug Induced Liver Injury (ortholog); Cole-Carpenter syndrome (ortholog); FOUND IN cytoskeleton (ortholog); cytosol (ortholog); endoplasmic reticulum (ortholog); INTERACTS WITH (S)-nicotine; 1,3-dinitrobenzene; 17alpha-ethynylestradiol | 10 | 106335300 | 106346911 | Rat | 325 | symbol , PhenoGen | gene, protein-coding, VALIDATED [RefSeq] |
| 11046 | P4hb | prolyl 4-hydroxylase, beta polypeptide | Enables integrin binding activity and protein-disulfide reductase activity. Involved in insulin processing. Acts upstream of with a positive effect on endoplasmic reticulum to Golgi vesicle-mediated transport. Acts upstream of or within cellular response to interleukin-7. Located in endoplasmic reti culum and external side of plasma membrane. Part of endoplasmic reticulum chaperone complex. Is active in endoplasmic reticulum lumen. Is expressed in several structures, including alimentary system; central nervous system; genitourinary system; heart; and sensory organ. Human ortholog(s) of this gene implicated in Cole-Carpenter syndrome. Orthologous to human P4HB (prolyl 4-hydroxylase subunit beta). [provided by Alliance of Genome Resources, Jul 2025] | 11 | 120451124 | 120464079 | Mouse | 331 | symbol , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 733481 | P4HB | prolyl 4-hydroxylase subunit beta | 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 prepro collagen. 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] | 17 | 81843166 | 81860535 | Human | 453 | symbol , COSMIC , Human Proteome Map , old_gene_symbol | gene, protein-coding, REVIEWED [RefSeq] |
| 8924243 | P4hb | prolyl 4-hydroxylase subunit beta | ENCODES a protein that exhibits actin binding (ortholog); enzyme binding (ortholog); integrin binding (ortholog); INVOLVED IN cellular response to hypoxia (ortholog); cellular response to interleukin-7 (ortholog); insulin processing (ortholog); ASSOCIATED WITH BCARD syndrome (ortholog); Chemical and Drug Induced Liver Injury (ortholog); Cole-Carpenter syndrome (ortholog); FOUND IN cytoskeleton (ortholog); cytosol (ortholog); endoplasmic reticulum (ortholog) | NW_004955506 | 1307932 | 1322011 | Chinchilla | 69 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12030507 | P4HB | prolyl 4-hydroxylase subunit beta | ENCODES a protein that exhibits enzyme binding (ortholog); protein disulfide isomerase activity (ortholog); protein-containing complex binding (ortholog); INVOLVED IN cellular response to interleukin-7 (ortholog); peptidyl-proline hydroxylation to 4-hydroxy-L-proline (ortholog); ASSOCIATED WITH BCAR D syndrome (ortholog); Chemical and Drug Induced Liver Injury (ortholog); Cole-Carpenter syndrome (ortholog); FOUND IN endoplasmic reticulum (ortholog); external side of plasma membrane (ortholog) | 17 | 76248252 | 76266152 | Bonobo | 76 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12237575 | P4HB | prolyl 4-hydroxylase subunit beta | ENCODES a protein that exhibits enzyme binding (ortholog); protein disulfide isomerase activity (ortholog); protein-containing complex binding (ortholog); INVOLVED IN cellular response to interleukin-7 (ortholog); peptidyl-proline hydroxylation to 4-hydroxy-L-proline (ortholog); ASSOCIATED WITH BCAR D syndrome (ortholog); Chemical and Drug Induced Liver Injury (ortholog); Cole-Carpenter syndrome (ortholog); FOUND IN cytoskeleton (inferred); cytosol (inferred); endoplasmic reticulum (inferred) | 9 | 427827 | 436880 | Dog | 72 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12507129 | P4hb | prolyl 4-hydroxylase subunit beta | ENCODES a protein that exhibits enzyme binding (ortholog); protein disulfide isomerase activity (ortholog); protein-containing complex binding (ortholog); INVOLVED IN cellular response to interleukin-7 (ortholog); peptidyl-proline hydroxylation to 4-hydroxy-L-proline (ortholog); ASSOCIATED WITH BCAR D syndrome (ortholog); Chemical and Drug Induced Liver Injury (ortholog); Cole-Carpenter syndrome (ortholog); FOUND IN endoplasmic reticulum (ortholog); external side of plasma membrane (ortholog) | NW_004936594 | 5368199 | 5379215 | Squirrel | 74 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 14157301 | P4HB | prolyl 4-hydroxylase subunit beta | ENCODES a protein that exhibits enzyme binding (ortholog); protein disulfide isomerase activity (ortholog); protein-containing complex binding (ortholog); INVOLVED IN cellular response to interleukin-7 (ortholog); peptidyl-proline hydroxylation to 4-hydroxy-L-proline (ortholog); ASSOCIATED WITH BCAR D syndrome (ortholog); Chemical and Drug Induced Liver Injury (ortholog); Cole-Carpenter syndrome (ortholog); FOUND IN cytoskeleton (inferred); cytosol (inferred); endoplasmic reticulum (inferred) | | | | Pig | 72 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18917071 | P4hb | prolyl 4-hydroxylase subunit beta | ENCODES a protein that exhibits actin binding (ortholog); enzyme binding (ortholog); integrin binding (ortholog); INVOLVED IN cellular response to hypoxia (ortholog); cellular response to interleukin-7 (ortholog); insulin processing (ortholog); ASSOCIATED WITH BCARD syndrome (ortholog); Chemical and Drug Induced Liver Injury (ortholog); Cole-Carpenter syndrome (ortholog); FOUND IN cytoskeleton (ortholog); cytosol (ortholog); endoplasmic reticulum (ortholog) | | | | Naked Mole-Rat | 73 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155236620 | p4hb | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 626081435 | P4hb | prolyl 4-hydroxylase subunit beta | ENCODES a protein that exhibits actin binding (ortholog); enzyme binding (ortholog); integrin binding (ortholog); INVOLVED IN cellular response to hypoxia (ortholog); cellular response to interleukin-7 (ortholog); insulin processing (ortholog); ASSOCIATED WITH BCARD syndrome (ortholog); Chemical and Drug Induced Liver Injury (ortholog); Cole-Carpenter syndrome (ortholog); FOUND IN cytoskeleton (ortholog); cytosol (ortholog); endoplasmic reticulum (ortholog) | | | | Black Rat | 69 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18711624 | P4HB | prolyl 4-hydroxylase subunit beta | ENCODES a protein that exhibits enzyme binding (ortholog); protein disulfide isomerase activity (ortholog); protein-containing complex binding (ortholog); INVOLVED IN cellular response to interleukin-7 (ortholog); peptidyl-proline hydroxylation to 4-hydroxy-L-proline (ortholog); ASSOCIATED WITH BCAR D syndrome (ortholog); Chemical and Drug Induced Liver Injury (ortholog); Cole-Carpenter syndrome (ortholog); FOUND IN endoplasmic reticulum (ortholog); external side of plasma membrane (ortholog) | | | | Green Monkey | 74 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155236621 | p4hb.L | | | | | | African Clawed Frog | | symbol | gene, null |
| 155238724 | p4hb.S | | | | | | African Clawed Frog | | symbol | gene, null |
| 597538570 | P4HB-AS1 | P4HB antisense RNA 1 | | | | | Human | | symbol , GTEx , COSMIC , name , Human Proteome Map | gene, ncrna |
| 16563631 | AC145207.3 | novel transcript, antisense P4HB | | | | | Human | | name | gene, lncrna |
