Literature DB >> 18337101

A patient with both Charcot-Marie-Tooth disease (CMT 1A) and mild spinal muscular atrophy (SMA 3).

Maria Jedrzejowska1, Barbara Ryniewicz, Dagmara Kabzińska, Hanna Drac, Irena Hausmanowa-Petrusewicz, Andrzej Kochański.   

Abstract

In the present study, we report a single Polish SMA family in which the 17p11.2-p12 duplication causative for the Charcot-Marie-Tooth type 1A disease (CMT1A) was found in addition to a deletion of exons 7 and 8 of the SMN1 gene. A patient harboring both SMA and CMT1A mutations manifested with SMA3 phenotype and foot deformity. Her electrophysiological testing showed chronic neurogenic changes in proximal muscles that are typical for SMA, but also slowed conduction velocity in motor and sensory fibers that is typical for demyelinating neuropathy.

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Year:  2008        PMID: 18337101     DOI: 10.1016/j.nmd.2008.02.001

Source DB:  PubMed          Journal:  Neuromuscul Disord        ISSN: 0960-8966            Impact factor:   4.296


  3 in total

Review 1.  Exploring the potential relevance of human-specific genes to complex disease.

Authors:  David N Cooper; Hildegard Kehrer-Sawatzki
Journal:  Hum Genomics       Date:  2011-01       Impact factor: 4.639

2.  Co-segregation of a homozygous SMN1 deletion and a heterozygous PMP22 duplication in a patient.

Authors:  Raquel M Fernández; Ana Peciña; Beatriz Muñoz-Cabello; Guillermo Antiñolo; Salud Borrego
Journal:  Clin Case Rep       Date:  2016-08-09

3.  UBA1/GARS-dependent pathways drive sensory-motor connectivity defects in spinal muscular atrophy.

Authors:  Hannah K Shorrock; Dinja van der Hoorn; Penelope J Boyd; Maica Llavero Hurtado; Douglas J Lamont; Brunhilde Wirth; James N Sleigh; Giampietro Schiavo; Thomas M Wishart; Ewout J N Groen; Thomas H Gillingwater
Journal:  Brain       Date:  2018-10-01       Impact factor: 13.501

  3 in total

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