P4HA1

Procollagen-proline, 2-oxoglutarate 4-dioxygenase (proline 4-hydroxylase), alpha polypeptide I
File:PBB Protein P4HA1 image.jpg PDB rendering based on 1tjc.
Available structures PDB Ortholog search: Template:Homologene2PDBe PDBe, Template:Homologene2uniprot RCSB List of PDB id codes 1tjc​
Identifiers
Symbols	P4HA1 ; 4-PH alpha-1; P4HA
External IDs	Template:OMIM5 Template:MGI HomoloGene: 30998
Gene ontology Molecular function Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Cellular component Template:GNF GO Biological process Template:GNF GO Template:GNF GO
RNA expression pattern
File:PBB GE P4HA1 207543 s at tn.png
File:PBB GE P4HA1 212537 x at tn.png
More reference expression data
Orthologs
Template:GNF Ortholog box
Species	Human	Mouse
Entrez	n/a	n/a
Ensembl	n/a	n/a
UniProt	n/a	n/a
RefSeq (mRNA)	n/a	n/a
RefSeq (protein)	n/a	n/a
Location (UCSC)	n/a	n/a
PubMed search	n/a	n/a

Procollagen-proline, 2-oxoglutarate 4-dioxygenase (proline 4-hydroxylase), alpha polypeptide I, also known as P4HA1, is a human gene.

This gene encodes a component of prolyl 4-hydroxylase, a key enzyme in collagen synthesis composed of two identical alpha subunits and two beta subunits. The encoded protein is one of several different types of alpha subunits and provides the major part of the catalytic site of the active enzyme. In collagen and related proteins, prolyl 4-hydroxylase catalyzes the formation of 4-hydroxyproline that is essential to the proper three-dimensional folding of newly synthesized procollagen chains. Alternatively spliced transcript variants encoding different isoforms have been described.

Further reading

• Helaakoski T, Vuori K, Myllylä R; et al. (1989). "Molecular cloning of the alpha-subunit of human prolyl 4-hydroxylase: the complete cDNA-derived amino acid sequence and evidence for alternative splicing of RNA transcripts". Proc. Natl. Acad. Sci. U.S.A. 86 (12): 4392–6. PMID 2543975.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Pajunen L, Jones TA, Helaakoski T; et al. (1990). "Assignment of the gene coding for the alpha-subunit of prolyl 4-hydroxylase to human chromosome region 10q21.3-23.1". Am. J. Hum. Genet. 45 (6): 829–34. PMID 2556027.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Helaakoski T, Veijola J, Vuori K; et al. (1994). "Structure and expression of the human gene for the alpha subunit of prolyl 4-hydroxylase. The two alternatively spliced types of mRNA correspond to two homologous exons the sequences of which are expressed in a variety of tissues". J. Biol. Chem. 269 (45): 27847–54. PMID 7961714.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Maruyama K, Sugano S (1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides". Gene. 138 (1–2): 171–4. PMID 8125298.
• Annunen P, Helaakoski T, Myllyharju J; et al. (1997). "Cloning of the human prolyl 4-hydroxylase alpha subunit isoform alpha(II) and characterization of the type II enzyme tetramer. The alpha(I) and alpha(II) subunits do not form a mixed alpha(I)alpha(II)beta2 tetramer". J. Biol. Chem. 272 (28): 17342–8. PMID 9211872.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K; et al. (1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library". Gene. 200 (1–2): 149–56. PMID 9373149.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Horelli-Kuitunen N, Kvist AP, Helaakoski T; et al. (1998). "The order and transcriptional orientation of the human COL13A1 and P4HA genes on chromosome 10 long arm determined by high-resolution FISH". Genomics. 46 (2): 299–302. doi:10.1006/geno.1997.5015. PMID 9417920.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Strausberg RL, Feingold EA, Grouse LH; et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. doi:10.1073/pnas.242603899. PMID 12477932.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Deloukas P, Earthrowl ME, Grafham DV; et al. (2004). "The DNA sequence and comparative analysis of human chromosome 10". Nature. 429 (6990): 375–81. doi:10.1038/nature02462. PMID 15164054.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Raveendran M, Senthil D, Utama B; et al. (2004). "Cigarette suppresses the expression of P4Halpha and vascular collagen production". Biochem. Biophys. Res. Commun. 323 (2): 592–8. doi:10.1016/j.bbrc.2004.08.129. PMID 15369792.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Gerhard DS, Wagner L, Feingold EA; et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMID 15489334.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Fähling M, Mrowka R, Steege A; et al. (2006). "Heterogeneous nuclear ribonucleoprotein-A2/B1 modulate collagen prolyl 4-hydroxylase, alpha (I) mRNA stability". J. Biol. Chem. 281 (14): 9279–86. doi:10.1074/jbc.M510925200. PMID 16464861.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Chen L, Shen YH, Wang X; et al. (2006). "Human prolyl-4-hydroxylase alpha(I) transcription is mediated by upstream stimulatory factors". J. Biol. Chem. 281 (16): 10849–55. doi:10.1074/jbc.M511237200. PMID 16488890.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Fähling M, Mrowka R, Steege A; et al. (2006). "Translational control of collagen prolyl 4-hydroxylase-alpha(I) gene expression under hypoxia". J. Biol. Chem. 281 (36): 26089–101. doi:10.1074/jbc.M604939200. PMID 16837461.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Grimmer C, Balbus N, Lang U; et al. (2006). "Regulation of type II collagen synthesis during osteoarthritis by prolyl-4-hydroxylases: possible influence of low oxygen levels". Am. J. Pathol. 169 (2): 491–502. PMID 16877351.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Koivunen P, Hirsilä M, Kivirikko KI, Myllyharju J (2006). "The length of peptide substrates has a marked effect on hydroxylation by the hypoxia-inducible factor prolyl 4-hydroxylases". J. Biol. Chem. 281 (39): 28712–20. doi:10.1074/jbc.M604628200. PMID 16885164.CS1 maint: Multiple names: authors list (link)