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pah-1 Phenylalanine-4-hydroxylase [ Caenorhabditis elegans ]

Gene ID: 174401, updated on 9-Dec-2024

Summary

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Official Symbol
pah-1
Official Full Name
Phenylalanine-4-hydroxylase
Primary source
WormBase:WBGene00000240
Locus tag
CELE_K08F8.4
See related
AllianceGenome:WB:WBGene00000240
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Caenorhabditis elegans (strain: Bristol N2)
Lineage
Eukaryota; Metazoa; Ecdysozoa; Nematoda; Chromadorea; Rhabditida; Rhabditina; Rhabditomorpha; Rhabditoidea; Rhabditidae; Peloderinae; Caenorhabditis
Summary
Enables phenylalanine 4-monooxygenase activity and tryptophan 5-monooxygenase activity. Involved in several processes, including aromatic amino acid metabolic process; collagen and cuticulin-based cuticle development; and determination of adult lifespan. Predicted to be located in cytoplasm. Is expressed in hypodermis and tail. Used to study phenylketonuria. Human ortholog(s) of this gene implicated in intellectual disability and phenylketonuria. Orthologous to human PAH (phenylalanine hydroxylase). [provided by Alliance of Genome Resources, Dec 2024]
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Genomic context

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See pah-1 in Genome Data Viewer
Location:
chromosome: II
Exon count:
10
Sequence:
Chromosome: II; NC_003280.10 (8762622..8766997, complement)

Chromosome II - NC_003280.10Genomic Context describing neighboring genes Neighboring gene ncRNA Neighboring gene Alpha-(1,3)-fucosyltransferase fut-1 Neighboring gene Nematode Specific Peptide family Neighboring gene Uncharacterized protein Neighboring gene ncRNA Neighboring gene Mediator of RNA polymerase II transcription subunit 13 Neighboring gene Sarcoglycan complex subunit protein

Genomic regions, transcripts, and products

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Genomic Sequence:
NC_003280.10

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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Related articles in PubMed

  1. A phenylalanine hydroxylase gene from the nematode C. elegans is expressed in the hypodermis. Loer CM, et al. J Neurogenet, 1999 Nov. PMID 10928216
  2. Superstoichiometric binding of L-Phe to phenylalanine hydroxylase from Caenorhabditis elegans: evolutionary implications. Flydal MI, et al. Amino Acids, 2010 Nov. PMID 20480196
  3. Anabolic function of phenylalanine hydroxylase in Caenorhabditis elegans. Calvo AC, et al. FASEB J, 2008 Aug. PMID 18460651
  4. Food and metabolic signalling defects in a Caenorhabditis elegans serotonin-synthesis mutant. Sze JY, et al. Nature, 2000 Feb 3. PMID 10676966
  5. Cuticle integrity and biogenic amine synthesis in Caenorhabditis elegans require the cofactor tetrahydrobiopterin (BH4). Loer CM, et al. Genetics, 2015 May. PMID 25808955, Free PMC Article

See all (20) citations in PubMed

GeneRIFs: Gene References Into Functions

Pathways from PubChem

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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 WormBase

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, 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 IDA
Inferred from Direct Assay
more info
 PubMed 
enables phenylalanine 4-monooxygenase activity IEA
Inferred from Electronic Annotation
more info
  
enables protein binding IPI
Inferred from Physical Interaction
more info
 PubMed 
enables tryptophan 5-monooxygenase activity IDA
Inferred from Direct Assay
more info
 PubMed 
enables tryptophan 5-monooxygenase activity IEA
Inferred from Electronic Annotation
more info
  
Process Evidence Code Pubs
involved_in L-phenylalanine catabolic process IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in L-phenylalanine catabolic process IEA
Inferred from Electronic Annotation
more info
  
involved_in aromatic amino acid metabolic process IEA
Inferred from Electronic Annotation
more info
  
involved_in collagen and cuticulin-based cuticle development IGI
Inferred from Genetic Interaction
more info
 PubMed 
involved_in determination of adult lifespan IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in melanin biosynthetic process IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in reproductive process IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in response to oxidative stress IGI
Inferred from Genetic Interaction
more info
 PubMed 
involved_in response to oxidative stress IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in tryptophan catabolic process IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in tyrosine biosynthetic process IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in tyrosine biosynthetic process IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in tyrosine biosynthetic process, by oxidation of phenylalanine IEA
Inferred from Electronic Annotation
more info
  
Component Evidence Code Pubs
located_in cytoplasm IEA
Inferred from Electronic Annotation
more info
  

General protein information

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Preferred Names
Phenylalanine-4-hydroxylase
NP_001254184.1
  • Confirmed by transcript evidence
NP_001254185.1
  • Confirmed by transcript evidence

NCBI Reference Sequences (RefSeq)

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Genome Annotation

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

Reference assembly

Genomic

  1. NC_003280.10 Reference assembly

    Range
    8762622..8766997 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_001267256.2 → NP_001254185.1  Phenylalanine-4-hydroxylase [Caenorhabditis elegans]

    See identical proteins and their annotated locations for NP_001254185.1

    Status: REVIEWED

    UniProtKB/Swiss-Prot
    I2HA98, P90925, Q9XYQ5
    Conserved Domains (3) summary
    TIGR01268
    Location:15 → 452
    Phe4hydrox_tetr; phenylalanine-4-hydroxylase, tetrameric form
    cd04904
    Location:30 → 99
    ACT_AAAH; ACT domain of the nonheme iron-dependent, aromatic amino acid hydroxylases (AAAH)
    pfam00351
    Location:119 → 448
    Biopterin_H; Biopterin-dependent aromatic amino acid hydroxylase

  2. NM_001267255.3 → NP_001254184.1  Phenylalanine-4-hydroxylase [Caenorhabditis elegans]

    See identical proteins and their annotated locations for NP_001254184.1

    Status: REVIEWED

    UniProtKB/TrEMBL
    I2HA98
    Conserved Domains (3) summary
    TIGR01268
    Location:11 → 448
    Phe4hydrox_tetr; phenylalanine-4-hydroxylase, tetrameric form
    cd04904
    Location:26 → 95
    ACT_AAAH; ACT domain of the nonheme iron-dependent, aromatic amino acid hydroxylases (AAAH)
    pfam00351
    Location:115 → 444
    Biopterin_H; Biopterin-dependent aromatic amino acid hydroxylase
Nucleotide Protein
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
P90925.2

Gene LinkOut

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