Literature DB >> 1355417

Variable dystrophin expression in different muscles of a Duchenne muscular dystrophy carrier.

F Muntoni1, A Mateddu, M G Marrosu, M Cau, R Congiu, M A Melis, A Cao, C Cianchetti.   

Abstract

The majority of Duchenne muscular dystrophy (DMD) female carriers show dystrophin immunostaining abnormalities, although a significant proportion of clinically non-manifesting carriers are normal following this analysis. We had the opportunity to study dystrophin immunostaining in two different muscles, the vastus lateralis and the rectus abdominis of a possible DMD carrier. While the vastus showed normal dystrophin immunostaining, pathological staining was detected in her rectus abdominis. These findings seem to indicate that dystrophin expression can vary in different muscle groups of a DMD carrier. The implications of these findings in DMD carrier detection and possible dystrophin function are discussed.

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Year:  1992        PMID: 1355417     DOI: 10.1111/j.1399-0004.1992.tb03133.x

Source DB:  PubMed          Journal:  Clin Genet        ISSN: 0009-9163            Impact factor:   4.438


  4 in total

1.  Clinical and genetic characterization of manifesting carriers of DMD mutations.

Authors:  Payam Soltanzadeh; Michael J Friez; Diane Dunn; Andrew von Niederhausern; Olga L Gurvich; Kathryn J Swoboda; Jacinda B Sampson; Alan Pestronk; Anne M Connolly; Julaine M Florence; Richard S Finkel; Carsten G Bönnemann; Livija Medne; Jerry R Mendell; Katherine D Mathews; Brenda L Wong; Michael D Sussman; Jonathan Zonana; Karen Kovak; Sidney M Gospe; Eduard Gappmaier; Laura E Taylor; Michael T Howard; Robert B Weiss; Kevin M Flanigan
Journal:  Neuromuscul Disord       Date:  2010-07-13       Impact factor: 4.296

2.  Integrated study of 100 patients with Xp21 linked muscular dystrophy using clinical, genetic, immunochemical, and histopathological data. Part 3. Differential diagnosis and prognosis.

Authors:  L V Nicholson; M A Johnson; K M Bushby; D Gardner-Medwin; A Curtis; I B Ginjaar; J T den Dunnen; J L Welch; T J Butler; E Bakker
Journal:  J Med Genet       Date:  1993-09       Impact factor: 6.318

3.  DMD carrier model with mosaic dystrophin expression in the heart reveals complex vulnerability to myocardial injury.

Authors:  Tatyana A Meyers; Jackie A Heitzman; DeWayne Townsend
Journal:  Hum Mol Genet       Date:  2020-04-15       Impact factor: 6.150

4.  Genetic characterization in symptomatic female DMD carriers: lack of relationship between X-inactivation, transcriptional DMD allele balancing and phenotype.

Authors:  Simona Brioschi; Francesca Gualandi; Chiara Scotton; Annarita Armaroli; Matteo Bovolenta; Maria S Falzarano; Patrizia Sabatelli; Rita Selvatici; Adele D'Amico; Marika Pane; Giulia Ricci; Gabriele Siciliano; Silvana Tedeschi; Antonella Pini; Liliana Vercelli; Domenico De Grandis; Eugenio Mercuri; Enrico Bertini; Luciano Merlini; Tiziana Mongini; Alessandra Ferlini
Journal:  BMC Med Genet       Date:  2012-08-16       Impact factor: 2.103

  4 in total

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