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pah phenylalanine hydroxylase [ Danio rerio (zebrafish) ]

Gene ID: 378962, updated on 11-Jun-2025
Official Symbol
pahprovided by ZNC
Official Full Name
phenylalanine hydroxylaseprovided by ZNC
Primary source
ZFIN:ZDB-GENE-031006-2
See related
Ensembl:ENSDARG00000020143 AllianceGenome:ZFIN:ZDB-GENE-031006-2
Gene type
protein coding
RefSeq status
PROVISIONAL
Organism
Danio rerio
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Actinopterygii; Neopterygii; Teleostei; Ostariophysi; Cypriniformes; Danionidae; Danioninae; Danio
Also known as
cb877; zgc:65981; wu:fb02f10
Summary
Predicted to enable phenylalanine 4-monooxygenase activity. Acts upstream of or within cellular response to estrogen stimulus. Is expressed in several structures, including digestive system; epidermis; eye; optic vesicle; and periderm. Human ortholog(s) of this gene implicated in intellectual disability and phenylketonuria. Orthologous to human PAH (phenylalanine hydroxylase). [provided by Alliance of Genome Resources, Jun 2025]
Orthologs
human mouse all
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See pah in Genome Data Viewer
Location:
chromosome: 4
Exon count:
13
Annotation release Status Assembly Chr Location
RS_2025_04 current GRCz12tu (GCF_049306965.1) 4 NC_133179.1 (17980184..17997946, complement)
RS_2024_08 previous assembly GRCz11 (GCF_000002035.6) 4 NC_007115.7 (17391680..17409367, complement)

Chromosome 4 - NC_133179.1Genomic Context describing neighboring genes Neighboring gene nucleoporin 37 Neighboring gene PARP1 binding protein Neighboring gene pro-melanin-concentrating hormone Neighboring gene tyrosine hydroxylase 2 Neighboring gene uncharacterized LOC101884203 Neighboring gene achaete-scute family bHLH transcription factor 1a

Go to nucleotide: Graphics FASTA GenBank

  • Project title: Sequencing the Zebrafish transcriptome from a range of tissues and developmental stages
  • Description: Sequencing the Zebrafish transcriptome from a range of tissues and developmental stages
  • BioProject: PRJEB1986
  • Analysis date: Fri Dec 8 19:48:10 2017

Related articles in PubMed

  1. Forward genetic analysis of visual behavior in zebrafish. Muto A, et al. PLoS Genet, 2005 Nov. PMID 16311625, Free PMC Article
  2. Two tyrosine hydroxylase genes in teleosts. Candy J, et al. Biochim Biophys Acta, 2005 Jan 21. PMID 15652156
  3. Zebrafish tyrosine hydroxylase 2 gene encodes tryptophan hydroxylase. Ren G, et al. J Biol Chem, 2013 Aug 2. PMID 23754283, Free PMC Article
  4. The toxic effect of triclosan and methyl-triclosan on biological pathways revealed by metabolomics and gene expression in zebrafish embryos. Fu J, et al. Ecotoxicol Environ Saf, 2020 Feb. PMID 31830605
  5. Transcriptome profiling of zebrafish infected with Streptococcus suis. Wu Z, et al. Microb Pathog, 2010 May. PMID 20206250

See all (19) citations in PubMed

GeneRIFs: Gene References Into Functions

Markers

Homology

Clone Names

  • MGC65981

Gene Ontology Provided by ZFIN

Function Evidence Code Pubs
enables iron ion binding IEA
Inferred from Electronic Annotation
more info
  
enables metal ion binding IEA
Inferred from Electronic Annotation
more info
  
enables monooxygenase activity 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, reduced pteridine as one donor, and incorporation of one atom of oxygen IEA
Inferred from Electronic Annotation
more info
  
enables phenylalanine 4-monooxygenase activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables phenylalanine 4-monooxygenase activity IEA
Inferred from Electronic Annotation
more info
  
Process Evidence Code Pubs
involved_in L-phenylalanine catabolic process IBA
Inferred from Biological aspect of Ancestor
more info
  
acts_upstream_of_or_within L-phenylalanine catabolic process IEA
Inferred from Electronic Annotation
more info
  
involved_in L-phenylalanine catabolic process IEA
Inferred from Electronic Annotation
more info
  
acts_upstream_of_or_within aromatic amino acid metabolic process IEA
Inferred from Electronic Annotation
more info
  
acts_upstream_of_or_within cellular response to estrogen stimulus IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in tyrosine biosynthetic process IBA
Inferred from Biological aspect of Ancestor
more info
  
Preferred Names
phenylalanine-4-hydroxylase
NP_956845.1

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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_200551.1 → NP_956845.1  phenylalanine-4-hydroxylase

    See identical proteins and their annotated locations for NP_956845.1

    Status: PROVISIONAL

    Source sequence(s)
    BC056537
    UniProtKB/TrEMBL
    Q6PHI7
    Related
    ENSDARP00000012808.6, ENSDART00000011943.7
    Conserved Domains (3) summary
    TIGR01268
    Location:20 → 449
    Phe4hydrox_tetr; phenylalanine-4-hydroxylase, tetrameric form
    cd04931
    Location:19 → 108
    ACT_PAH; ACT domain of the nonheme iron-dependent aromatic amino acid hydroxylase, phenylalanine hydroxylases (PAH)
    pfam00351
    Location:117 → 447
    Biopterin_H; Biopterin-dependent aromatic amino acid hydroxylase

RefSeqs of Annotated Genomes: GCF_049306965.1-RS_2025_04

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

Reference GRCz12tu Primary Assembly

Genomic

  1. NC_133179.1 Reference GRCz12tu Primary Assembly

    Range
    17980184..17997946 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)
Nucleotide Protein Strain
Heading Accession and Version

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