http://www.cnr.it/ontology/cnr/individuo/prodotto/ID10295
Parallel protein and transcript profiles of FSHD patient muscles correlate to the D4Z4 arrangement and reveal a common impairment of slow to fast fibre differentiation and a general deregulation of MyoD-dependent genes. (Articolo in rivista)
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- Parallel protein and transcript profiles of FSHD patient muscles correlate to the D4Z4 arrangement and reveal a common impairment of slow to fast fibre differentiation and a general deregulation of MyoD-dependent genes. (Articolo in rivista) (literal)
- Anno
- 2006-01-01T00:00:00+01:00 (literal)
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Celegato B. 1, Capitanio D. 2, Pescatori M. 3, Romualdi C. 1, Pacchioni B. 1, Cagnin S. 1, Vigano A. 2, Colantoni L. 3, Begum S. 4, Ricci E. 3, Wait R. 4, Lanfranchi G. 1, Gelfi C. 2 (2006)
Parallel protein and transcript profiles of FSHD patient muscles correlate to the D4Z4 arrangement and reveal a common impairment of slow to fast fibre differentiation and a general deregulation of MyoD-dependent genes.
in Proteomics (Weinh., Print)
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- Celegato B. 1, Capitanio D. 2, Pescatori M. 3, Romualdi C. 1, Pacchioni B. 1, Cagnin S. 1, Vigano A. 2, Colantoni L. 3, Begum S. 4, Ricci E. 3, Wait R. 4, Lanfranchi G. 1, Gelfi C. 2 (literal)
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- 1. CRIBI Biotechnology Centre and Department of Biology, Università degli Studi di Padova, Padova, Italy
2. Institute of Bioimaging and Molecular Physiology, CNR, Segrate, Milano, Italy
3. Department of Neurology, Università Cattolica del Sacro Cuore, Roma, Italy and Center for Neuromuscular diseases UILDM Sezione Laziale
4. The Kennedy Institute of Rheumatology Division, Imperial College, London, UK (literal)
- Titolo
- Parallel protein and transcript profiles of FSHD patient muscles correlate to the D4Z4 arrangement and reveal a common impairment of slow to fast fibre differentiation and a general deregulation of MyoD-dependent genes. (literal)
- Abstract
- Here, we present the first study of a human neuromuscular disorder at transcriptional and proteomic
level. Autosomal dominant facio-scapulo-humeral muscular dystrophy (FSHD) is caused
by a deletion of an integral number of 3.3-kb KpnI repeats inside the telomeric region D4Z4 at
the 4q35 locus. We combined a muscle-specific cDNA microarray platform with a proteomic
investigation to analyse muscle biopsies of patients carrying a variable number of KpnI repeats.
Unsupervised cluster analysis divides patients into three classes, according to their KpnI repeat
number. Expression data reveal a transition from fast-glycolytic to slow-oxidative phenotype in
FSHD muscle, which is accompanied by a deficit of proteins involved in response to oxidative
stress. Besides, FSHD individuals show a disruption in the MyoD-dependent gene network
suggesting a coregulation at transcriptional level during myogenesis. We also discuss the hypothesis
that D4Z4 contraction may affect in trans the expression of a set of genes involved in
myogenesis, as well as in the regeneration pathway of satellite cells in adult tissue. Muscular
wasting could result from the inability of satellite cells to successfully differentiate into mature
fibres and from the accumulation of structural damages caused by a reactive oxygen species
(ROS) imbalance induced by an increased oxidative metabolism in fibres. (literal)
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