Literature DB >> 16924035

Do carriers of PYGM mutations have symptoms of McArdle disease?

Susanne Tvede Andersen1, Morten Dunø, Marianne Schwartz, John Vissing.   

Abstract

The authors investigated whether carriers of single myophosphorylase gene (PYGM) mutations have symptoms of McArdle disease. They studied the oxidative capacity and lactate responses to maximal cycle exercise in eight patients with McArdle disease, seven single PYGM mutation carriers, and 11 healthy subjects. Heterozygotes had maximal oxidative capacity and peak lactate responses identical to control subjects. Thus, carriers of single PYGM mutations are not prone to develop symptoms of McArdle disease.

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Year:  2006        PMID: 16924035     DOI: 10.1212/01.wnl.0000230154.79933.d7

Source DB:  PubMed          Journal:  Neurology        ISSN: 0028-3878            Impact factor:   9.910


  4 in total

1.  Phenotype consequences of myophosphorylase dysfunction: insights from the McArdle mouse model.

Authors:  Astrid Brull; Noemí de Luna; Albert Blanco-Grau; Alejandro Lucia; Miguel Angel Martin; Joaquin Arenas; Ramon Martí; Antoni L Andreu; Tomàs Pinós
Journal:  J Physiol       Date:  2015-05-18       Impact factor: 5.182

Review 2.  Rodent models for resolving extremes of exercise and health.

Authors:  Fleur C Garton; Kathryn N North; Lauren G Koch; Steven L Britton; Gisela Nogales-Gadea; Alejandro Lucia
Journal:  Physiol Genomics       Date:  2015-09-22       Impact factor: 3.107

3.  Manifesting heterozygotes in McArdle disease: a myth or a reality-role of statins.

Authors:  Judit Núñez-Manchón; Alfonsina Ballester-Lopez; Emma Koehorst; Ian Linares-Pardo; Daniëlle Coenen; Ignacio Ara; Carlos Rodriguez-Lopez; Alba Ramos-Fransi; Alicia Martínez-Piñeiro; Giuseppe Lucente; Miriam Almendrote; Jaume Coll-Cantí; Guillem Pintos-Morell; Alejandro Santos-Lozano; Joaquin Arenas; Miguel Angel Martín; Mauricio de Castro; Alejandro Lucia; Alfredo Santalla; Gisela Nogales-Gadea
Journal:  J Inherit Metab Dis       Date:  2018-06-20       Impact factor: 4.982

Review 4.  The hereditary inclusion body myopathy enigma and its future therapy.

Authors:  Zohar Argov; Stella Mitrani-Rosenbaum
Journal:  Neurotherapeutics       Date:  2008-10       Impact factor: 7.620

  4 in total

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