Prolyl 4-hydroxylase subunit alpha-2 precursor

Names and origin

Protein attributes

General annotation (Comments)

Ontologies

Binary interactions

Alternative products

Sequence annotation (Features)

Sequences

References

Cross-references

Entry information

Relevant documents

Names and origin

Protein attributes

General annotation (Comments)

Ontologies

Binary interactions

Alternative products

Sequence annotation (Features)

Sequences

References

Cross-references

Entry information

Relevant documents

Molecule processing

Regions

Sites

Amino acid modifications

Natural variations

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Entry

#Exp.

IntAct

Notes

Molecule processing

Regions

Sites

Amino acid modifications

Natural variations

Sequence databases

3D structure databases

Protein-protein interaction databases

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Reviewed, UniProtKB/Swiss-Prot O15460 (P4HA2_HUMAN)

Last modified July 7, 2009. Version 90. History...

Clusters with 100%, 90%, 50% identity |

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Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Binary interactions · Alternative products · Sequence annotation (Features) · Sequences · References · Cross-references · Entry information · Relevant documents

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Protein names

Recommended name:
    Prolyl 4-hydroxylase subunit alpha-2
    EC=1.14.11.2
Alternative name(s):
    4-PH alpha-2
    Procollagen-proline,2-oxoglutarate-4-dioxygenase subunit alpha-2

Gene names

Name:	P4HA2
ORF Names:	UNQ290/PRO330

Organism

Homo sapiens (Human) [Complete proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

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Sequence length	535 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at protein level.

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Function	Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
Catalytic activity	Procollagen L-proline + 2-oxoglutarate + O₂ = procollagen trans-4-hydroxy-L-proline + succinate + CO₂.
Cofactor	Binds 1 Fe²⁺ ion per subunit By similarity. Ascorbate By similarity.
Subunit structure	Heterotetramer of two alpha-2 chains and two beta chains (the beta chain is the multi-functional PDI).
Subcellular location	Endoplasmic reticulum lumen.
Sequence similarities	Belongs to the P4HA family. Contains 1 PKHD (prolyl/lysyl hydroxylase) domain. Contains 1 TPR repeat.

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Keywords
Cellular component	Endoplasmic reticulum
Coding sequence diversity	Alternative splicing
Domain	Signal TPR repeat
Ligand	Iron Metal-binding Vitamin C
Molecular function	Dioxygenase Oxidoreductase
PTM	Glycoprotein
Technical term	Complete proteome
Gene Ontology (GO)
Biological process	oxidation reduction Inferred from electronic annotation. Source: UniProtKB-KW
Cellular component	endoplasmic reticulum Inferred from electronic annotation. Source: UniProtKB-KW endoplasmic reticulum lumen Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular function	L-ascorbic acid binding Inferred from electronic annotation. Source: UniProtKB-KW electron carrier activity Ref.1 Traceable author statement. Source: UniProtKB iron ion binding Inferred from electronic annotation. Source: UniProtKB-KW oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen Inferred from electronic annotation. Source: UniProtKB-KW procollagen-proline 4-dioxygenase activity Ref.1 Traceable author statement. Source: ProtInc protein binding Inferred from physical interaction. Source: IntAct
Complete GO annotation...

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With	Entry	#Exp.	IntAct	Notes
BAT2	P48634	1	EBI-348033,EBI-347545

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Feature key

Position(s)

Length

Description

Graphical view

Feature identifier

Signal peptide

1 – 21

Potential

Chain

22 – 535

514

Prolyl 4-hydroxylase subunit alpha-2

PRO_0000022726

Repeat

207 – 240

TPR

Domain

336 – 519

184

PKHD

Metal binding

430

Iron By similarity

Metal binding

432

Iron By similarity

Metal binding

501

Iron By similarity

Binding site

511

2-oxoglutarate Potential

Glycosylation

115

N-linked (GlcNAc...) Potential

Glycosylation

264

N-linked (GlcNAc...) Potential

Alternative sequence

436 – 451

NDERD…TGNRV → RPFDSGLKTEGNRL in isoform IIa.

VSP_004506

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Sequence		Length	Mass (Da)
Isoform IIb [UniParc]. Last modified January 1, 1998. Version 1. Checksum: FD04467B098F63CF Show »	FASTA	535	60,902
10 20 30 40 50 60 MKLWVSALLM AWFGVLSCVQ AEFFTSIGHM TDLIYAEKEL VQSLKEYILV EEAKLSKIKS 70 80 90 100 110 120 WANKMEALTS KSAADAEGYL AHPVNAYKLV KRLNTDWPAL EDLVLQDSAA GFIANLSVQR 130 140 150 160 170 180 QFFPTDEDEI GAAKALMRLQ DTYRLDPGTI SRGELPGTKY QAMLSVDDCF GMGRSAYNEG 190 200 210 220 230 240 DYYHTVLWME QVLKQLDAGE EATTTKSQVL DYLSYAVFQL GDLHRALELT RRLLSLDPSH 250 260 270 280 290 300 ERAGGNLRYF EQLLEEEREK TLTNQTEAEL ATPEGIYERP VDYLPERDVY ESLCRGEGVK 310 320 330 340 350 360 LTPRRQKRLF CRYHHGNRAP QLLIAPFKEE DEWDSPHIVR YYDVMSDEEI ERIKEIAKPK 370 380 390 400 410 420 LARATVRDPK TGVLTVASYR VSKSSWLEED DDPVVARVNR RMQHITGLTV KTAELLQVAN 430 440 450 460 470 480 YGVGGQYEPH FDFSRNDERD TFKHLGTGNR VATFLNYMSD VEAGGATVFP DLGAAIWPKK 490 500 510 520 530 GTAVFWYNLL RSGEGDYRTR HAACPVLVGC KWVSNKWFHE RGQEFLRPCG STEVD « Hide
Isoform IIa. Checksum: 8C875AD482B0DBD2 Show »	FASTA	533	60,633
10 20 30 40 50 60 MKLWVSALLM AWFGVLSCVQ AEFFTSIGHM TDLIYAEKEL VQSLKEYILV EEAKLSKIKS 70 80 90 100 110 120 WANKMEALTS KSAADAEGYL AHPVNAYKLV KRLNTDWPAL EDLVLQDSAA GFIANLSVQR 130 140 150 160 170 180 QFFPTDEDEI GAAKALMRLQ DTYRLDPGTI SRGELPGTKY QAMLSVDDCF GMGRSAYNEG 190 200 210 220 230 240 DYYHTVLWME QVLKQLDAGE EATTTKSQVL DYLSYAVFQL GDLHRALELT RRLLSLDPSH 250 260 270 280 290 300 ERAGGNLRYF EQLLEEEREK TLTNQTEAEL ATPEGIYERP VDYLPERDVY ESLCRGEGVK 310 320 330 340 350 360 LTPRRQKRLF CRYHHGNRAP QLLIAPFKEE DEWDSPHIVR YYDVMSDEEI ERIKEIAKPK 370 380 390 400 410 420 LARATVRDPK TGVLTVASYR VSKSSWLEED DDPVVARVNR RMQHITGLTV KTAELLQVAN 430 440 450 460 470 480 YGVGGQYEPH FDFSRRPFDS GLKTEGNRLA TFLNYMSDVE AGGATVFPDL GAAIWPKKGT 490 500 510 520 530 AVFWYNLLRS GEGDYRTRHA ACPVLVGCKW VSNKWFHERG QEFLRPCGST EVD « Hide

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	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"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." Annunen P., Helaakoski T., Myllyharju J., Veijola J., Pihlajaniemi T., Kivirikko K.I. J. Biol. Chem. 272:17342-17348(1997) [PubMed: 9211872] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM IIB). Tissue: Lung.
[2]	"Characterization of the human and mouse genes for the alpha subunit of type II prolyl 4-hydroxylase. Identification of a previously unknown alternatively spliced exon and its expression in various tissues." Nokelainen M., Nissi R., Kukkola L., Helaakoski T., Myllyharju J. Eur. J. Biochem. 268:5300-5309(2001) [PubMed: 11606192] [Abstract] Cited for: NUCLEOTIDE SEQUENCE (ISOFORMS IIA AND IIB).
[3]	"The secreted protein discovery initiative (SPDI), a large-scale effort to identify novel human secreted and transmembrane proteins: a bioinformatics assessment." Clark H.F., Gurney A.L., Abaya E., Baker K., Baldwin D.T., Brush J., Chen J., Chow B., Chui C., Crowley C., Currell B., Deuel B., Dowd P., Eaton D., Foster J.S., Grimaldi C., Gu Q., Hass P.E. Gray A.M. Genome Res. 13:2265-2270(2003) [PubMed: 12975309] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM IIA).
[4]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM IIA). Tissue: Brain.
[5]	Colinge J., Superti-Furga G., Bennett K.L. Submitted (OCT-2008) to UniProtKB Cited for: IDENTIFICATION [LARGE SCALE ANALYSIS], MASS SPECTROMETRY.
+	Additional computationally mapped references.

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Sequence databases
	U90441 mRNA. Translation: AAB71339.1. AJ314859 Genomic DNA. Translation: CAC85688.1. AJ314859 Genomic DNA. Translation: CAC85689.1. AY358970 mRNA. Translation: AAQ89329.1. BC035813 mRNA. Translation: AAH35813.1.
IPI	IPI00003128. IPI00182373.
RefSeq	NP_001017973.1. NP_001017974.1. NP_001136071.1. NP_004190.1.
UniGene	Hs.519568
3D structure databases
ModBase	Search...
Protein-protein interaction databases
IntAct	O15460. 1 interaction.
Proteomic databases
PRIDE	O15460.
Genome annotation databases
Ensembl	ENSG00000072682. Homo sapiens. [Contig view]
GeneID	8974.
KEGG	hsa:8974.
UCSC	uc003kwh.1. human. uc003kwj.1. human.
Organism-specific databases
GeneCards	GC05M131556.
H-InvDB	HIX0024833.
HGNC	HGNC:8547. P4HA2.
MIM	600608. gene.
PharmGKB	PA32875.
GenAtlas	Search...
Phylogenomic databases
HOGENOM	O15460.
HOVERGEN	O15460.
OMA	O15460. SNKWFHE.
Enzyme and pathway databases
BRENDA	1.14.11.2. 247.
Gene expression databases
ArrayExpress	O15460.
Bgee	O15460.
CleanEx	HS_P4HA2.
GermOnline	ENSG00000072682. Homo sapiens.
Family and domain databases
InterPro	IPR005123. Oxoglutarate/Fe-dep_Oase. IPR006620. Pro_4_hyd_alph. IPR013547. Pro_4_hyd_alph_N. IPR011990. TPR-like_helical. IPR013026. TPR_region. IPR019734. TPR_repeat. [Graphical view]
Gene3D	G3DSA:1.25.40.10. TPR-like_helical. 1 hit.
Pfam	PF03171. 2OG-FeII_Oxy. 1 hit. PF08336. P4Ha_N. 1 hit. [Graphical view]
SMART	SM00702. P4Hc. 1 hit. [Graphical view]
PROSITE	PS50005. TPR. 1 hit. PS50293. TPR_REGION. 1 hit. [Graphical view]
ProtoNet	Search...
Other Resources
DrugBank	DB00172. L-Proline. DB00139. Succinic acid.
NextBio	33677.
SOURCE	Search...

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Entry name

P4HA2_HUMAN

Accession

Primary (citable) accession number: O15460
Secondary accession number(s): Q8WWN0

Entry history

Integrated into UniProtKB/Swiss-Prot:	May 2, 2002
Last sequence update:	January 1, 1998
Last modified:	July 7, 2009
This is version 90 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)

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Human chromosome 5

Human chromosome 5: entries, gene names and cross-references to MIM

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

SIMILARITY comments

Index of protein domains and families

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]

Isoform IIb (identifier: O15460-1)

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

Isoform IIa (identifier: O15460-2)

The sequence of this isoform differs from the canonical sequence as follows:
436-451: NDERDTFKHLGTGNRV → RPFDSGLKTEGNRL