PHEX

phosphate regulating endopeptidase homolog X-linked
OMIM: 300550, Gene2Phenotype

8 panels

Panel Reviews Mode of inheritance Details
8 panels
Green PHEX in Hypophosphataemia or rickets


Level 2: Endocrinology
Version 4.3
Latest signed off version: v4.2 (6 May 2026)

review X-LINKED: hemizygous mutation in males, monoallelic mutations in females may cause disease (may be less severe, later onset than males)
Sources
  • Expert Review Green
  • Radboud University Medical Center, Nijmegen
  • Illumina TruGenome Clinical Sequencing Services
  • Emory Genetics Laboratory
  • UKGTN
  • Expert list
Phenotypes
  • Hypophosphatemic rickets, X-linked dominant (307800)
Green PHEX in Nephrocalcinosis or nephrolithiasis


Level 2: Renal
Version 6.1
Latest signed off version: v6.0 (6 May 2026)

Component of the following Super Panels:

  • Unexplained young onset end-stage renal disease
  • review X-LINKED: hemizygous mutation in males, monoallelic mutations in females may cause disease (may be less severe, later onset than males)
    Sources
    • Expert Review Green
    • Literature
    • Expert list
    Phenotypes
    • Hypophosphatemic rickets, X-linked dominant 307800
    Green PHEX in Skeletal dysplasia


    Level 2: Musculoskeletal
    Version 9.1
    Latest signed off version: v9.0 (6 May 2026)

    Component of the following Super Panels:

  • Paediatric disorders
  • review X-LINKED: hemizygous mutation in males, monoallelic mutations in females may cause disease (may be less severe, later onset than males)
    Sources
    • NHS GMS
    • Expert Review Green
    • Radboud University Medical Center, Nijmegen
    • UKGTN
    • Expert list
    • Illumina TruGenome Clinical Sequencing Services
    • Emory Genetics Laboratory
    • Expert
    Phenotypes
    • Hypophosphatemic rickets, X-linked dominant 307800
    Amber PHEX in Fetal anomalies


    Level 2: Fetal (including NIPD)
    Version 7.1
    Latest signed off version: v7.0 (6 May 2026)

    review X-LINKED: hemizygous mutation in males, monoallelic mutations in females may cause disease (may be less severe, later onset than males)
    Sources
    • NHS GMS
    • Expert Review Amber
    Phenotypes
    • Hypophosphatemic rickets, X-linked dominant, OMIM:307800
    Green PHEX in Rare syndromic craniosynostosis or isolated multisuture synostosis


    Level 2: Musculoskeletal
    Version 6.5
    Latest signed off version: v6.0 (30 Apr 2025)

    review X-LINKED: hemizygous mutation in males, monoallelic mutations in females may cause disease (may be less severe, later onset than males)
    Sources
    • NHS GMS
    • Expert Review Green
    • Expert Review
    Phenotypes
    • X-linked hypophosphataemic rickets
    Red PHEX in Osteogenesis imperfecta


    Level 2: Musculoskeletal
    Version 6.1
    Latest signed off version: v6.0 (6 May 2026)

    review Not set
    Sources
    • Expert Review Red
    • Emory Genetics Laboratory
    • Expert
    Phenotypes
    • Osteogenesis Imperfecta and Decreased Bone Density
    • skeletal dysplasias
    Green PHEX in DDG2P


    Version 7.1
    Latest signed off version: v7.0 (6 May 2026)

    Component of the following Super Panels:

  • Paediatric disorders
  • review X-LINKED: hemizygous mutation in males, monoallelic mutations in females may cause disease (may be less severe, later onset than males)
    Sources
    • DD-Gene2Phenotype
    • Expert Review Green
    Phenotypes
    • MONDO:0010619
    • PHEX-related hypophosphatemic rickets
    • OMIM:307800.0
    Red PHEX in Monogenic hearing loss


    Level 2: Audiology
    Version 6.1
    Latest signed off version: v6.0 (6 May 2026)

    Component of the following Super Panels:

  • Paediatric disorders
  • review Not set
    Sources
    • Expert