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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

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