• Medientyp: E-Artikel
  • Titel: Two novel mutations in the muscle glycogen phosphorylase gene in McArdle's disease
  • Beteiligte: Gámez, Josep; Rubio, Juan C.; Martín, Miguel A.; Fernández‐Cadenas, Israel; Garcia‐Arumi, Elena; Andreu, Antoni L.; Arenas, Joaquín
  • Erschienen: Wiley, 2003
  • Erschienen in: Muscle & Nerve
  • Sprache: Englisch
  • DOI: 10.1002/mus.10418
  • ISSN: 0148-639X; 1097-4598
  • Schlagwörter: Physiology (medical) ; Cellular and Molecular Neuroscience ; Neurology (clinical) ; Physiology
  • Entstehung:
  • Anmerkungen:
  • Beschreibung: <jats:title>Abstract</jats:title><jats:p>We report on a Spanish family with myophosphorylase (EC 2.4.1.1) deficiency (McArdle's disease). The proband and his symptomatic sister were compound heterozygous for two novel mutations: a T‐to‐G transversion in exon 14 (c1722 T&gt;G) that changes a tyrosine to a stop codon (Y573X), and a G‐to‐A transition in exon 15 (c1827 G&gt;A) that disrupts the consensus signal at the donor splicing site. These findings further expand knowledge of the genetic bases of muscle glycogen phosphorylase deficiency. Muscle Nerve 28: 380–382, 2003</jats:p>