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P3h3 prolyl 3-hydroxylase 3 [ Mus musculus (house mouse) ]

Gene ID: 14789, updated on 25-Apr-2024

Summary

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Official Symbol
P3h3provided by MGI
Official Full Name
prolyl 3-hydroxylase 3provided by MGI
Primary source
MGI:MGI:1315208
See related
Ensembl:ENSMUSG00000023191 AllianceGenome:MGI:1315208
Gene type
protein coding
RefSeq status
VALIDATED
Organism
Mus musculus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as
Grcb; Leprel2
Summary
Predicted to enable procollagen-proline 3-dioxygenase activity. Involved in collagen biosynthetic process and peptidyl-lysine hydroxylation. Part of catalytic complex. Is expressed in several structures, including branchial arch; embryo mesenchyme; genitourinary system; limb primordium; and sensory organ. Orthologous to human P3H3 (prolyl 3-hydroxylase 3). [provided by Alliance of Genome Resources, Apr 2022]
Expression
Broad expression in limb E14.5 (RPKM 62.5), ovary adult (RPKM 28.8) and 18 other tissues See more
Orthologs
human all
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Genomic context

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Location:
6 F2; 6 59.17 cM
Exon count:
15
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCm39 (GCF_000001635.27) 6 NC_000072.7 (124818052..124834680, complement)
108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 6 NC_000072.6 (124841089..124857746, complement)

Chromosome 6 - NC_000072.7Genomic Context describing neighboring genes Neighboring gene cell division cycle associated 3 Neighboring gene guanine nucleotide binding protein (G protein), beta 3 Neighboring gene STARR-positive B cell enhancer ABC_E6487 Neighboring gene predicted gene, 32404 Neighboring gene CapStarr-seq enhancer MGSCv37_chr6:124812982-124813183 Neighboring gene G protein-coupled receptor 162 Neighboring gene CapStarr-seq enhancer MGSCv37_chr6:124814119-124814302 Neighboring gene CD4 antigen Neighboring gene CapStarr-seq enhancer MGSCv37_chr6:124829493-124829732 Neighboring gene CapStarr-seq enhancer MGSCv37_chr6:124836126-124836313

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: Mouse ENCODE transcriptome data
  • Description: RNA profiling data sets generated by the Mouse ENCODE project.
  • BioProject: PRJNA66167
  • Publication: PMID 25409824
  • Analysis date: n/a

Bibliography

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Related articles in PubMed

  1. Type I and type V procollagen triple helix uses different subsets of the molecular ensemble for lysine posttranslational modifications in the rER. Ishikawa Y, et al. J Biol Chem, 2021 Jan-Jun. PMID 33631195, Free PMC Article
  2. Expression of prolyl 3-hydroxylase genes in embryonic and adult mouse tissues. Vranka J, et al. Cell Struct Funct, 2009. PMID 19652424
  3. P3h3-null and Sc65-null Mice Phenocopy the Collagen Lysine Under-hydroxylation and Cross-linking Abnormality of Ehlers-Danlos Syndrome Type VIA. Hudson DM, et al. J Biol Chem, 2017 Mar 3. PMID 28115524, Free PMC Article
  4. LEPREL1, a novel ER and Golgi resident member of the Leprecan family. Järnum S, et al. Biochem Biophys Res Commun, 2004 Apr 30. PMID 15063763
  5. Prolyl 3-hydroxylase 1, enzyme characterization and identification of a novel family of enzymes. Vranka JA, et al. J Biol Chem, 2004 May 28. PMID 15044469

See all (25) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Type I and type V procollagen triple helix uses different subsets of the molecular ensemble for lysine posttranslational modifications in the rER.
    Title: Type I and type V procollagen triple helix uses different subsets of the molecular ensemble for lysine posttranslational modifications in the rER.
  2. both P3h3- and Sc65-null mice displayed a similar pattern of severe under-hydroxylation of lysines, particularly at cross-linking sites in type I collagen from bone, skin, dentin, cornea, and aorta, revealing a complex evolution and interplay between the proteins forming the machinery of collagen prolyl 3-hydroxylation and lysyl hydroxylation in the ER
    Title: P3h3-null and Sc65-null Mice Phenocopy the Collagen Lysine Under-hydroxylation and Cross-linking Abnormality of Ehlers-Danlos Syndrome Type VIA.
  3. This report characterizes expression of the P3H1, P3H2 and P3H3 genes in embryonic and adult mice.
    Title: Expression of prolyl 3-hydroxylase genes in embryonic and adult mouse tissues.
  4. Study found that all 3 mouse Leprecan genes Lepre1, leprel1 and leprel2 are expressed in the notochord; additionally, they are expressed in dorsal root ganglia, limb buds, branchial arches, and developing kidneys.
    Title: Conservation of notochord gene expression across chordates: insights from the Leprecan gene family.
Submit: New GeneRIF Correction

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics  (MGI)
  • Endonuclease-mediated (1) 
  • Targeted (3)  1 citation

Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Markers

Homology

Gene Ontology Provided by MGI

Function Evidence Code Pubs
enables L-ascorbic acid binding IEA
Inferred from Electronic Annotation
more info
  
enables dioxygenase activity IEA
Inferred from Electronic Annotation
more info
  
enables iron ion binding IEA
Inferred from Electronic Annotation
more info
  
enables metal ion binding IEA
Inferred from Electronic Annotation
more info
  
enables oxidoreductase activity IEA
Inferred from Electronic Annotation
more info
  
enables oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen IEA
Inferred from Electronic Annotation
more info
  
enables procollagen-proline 3-dioxygenase activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables protein binding IPI
Inferred from Physical Interaction
more info
 PubMed 
Process Evidence Code Pubs
involved_in collagen biosynthetic process IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in collagen metabolic process IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in negative regulation of cell population proliferation ISO
Inferred from Sequence Orthology
more info
  
involved_in peptidyl-lysine hydroxylation IMP
Inferred from Mutant Phenotype
more info
 PubMed 
Component Evidence Code Pubs
part_of catalytic complex IMP
Inferred from Mutant Phenotype
more info
 PubMed 
is_active_in endoplasmic reticulum IBA
Inferred from Biological aspect of Ancestor
more info
  

General protein information

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Preferred Names
prolyl 3-hydroxylase 3
Names
gene rich cluster, B
leprecan-like protein 2
protein B
NP_038562.2

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

  1. NM_013534.5 → NP_038562.2  prolyl 3-hydroxylase 3 precursor

    See identical proteins and their annotated locations for NP_038562.2

    Status: VALIDATED

    Source sequence(s)
    AC142254
    Consensus CDS
    CCDS20533.1
    UniProtKB/Swiss-Prot
    O88836, Q8CG70
    Related
    ENSMUSP00000023958.4, ENSMUST00000023958.10
    Conserved Domains (1) summary
    smart00702
    Location:470 → 670
    P4Hc; Prolyl 4-hydroxylase alpha subunit homologues

RNA

  1. NR_163804.1 RNA Sequence

    Status: VALIDATED

    Source sequence(s)
    AC142254
    Related
    ENSMUST00000135127.8

  2. NR_163805.1 RNA Sequence

    Status: VALIDATED

    Source sequence(s)
    AC142254

  3. NR_163806.1 RNA Sequence

    Status: VALIDATED

    Source sequence(s)
    AC142254

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

  1. NC_000072.7 Reference GRCm39 C57BL/6J

    Range
    124818052..124834680 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)
Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q8CG70.1 GenPept UniProtKB/Swiss-Prot:Q8CG70

Gene LinkOut

The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

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