Chapter title |
Proteomic Profiling of the Dystrophin-Deficient Brain
|
---|---|
Chapter number | 7 |
Book title |
Duchenne Muscular Dystrophy
|
Published in |
Methods in molecular biology, January 2018
|
DOI | 10.1007/978-1-4939-7374-3_7 |
Pubmed ID | |
Book ISBNs |
978-1-4939-7373-6, 978-1-4939-7374-3
|
Authors |
Sandra Murphy, Kay Ohlendieck, Murphy, Sandra, Ohlendieck, Kay |
Abstract |
Duchenne muscular dystrophy is a highly progressive neuromuscular disorder caused by primary abnormalities in the Dmd gene encoding the membrane cytoskeletal protein dystrophin. Dystrophinopathies are multi-systems disorders that are characterized by severe skeletal muscle wasting, with loss of independent ambulation in the early teenage years, followed by cardio-respiratory complications and premature death. Nonprogressive cognitive impairments are estimated to affect approximately one-third of dystrophic children. To identify the molecular mechanisms behind the impaired brain function in dystrophinopathy, liquid chromatography-based mass spectrometry offers an unbiased and technology-driven approach. In this chapter, we give a detailed description of a label-free mass spectrometric method to investigate proteome-wide changes in the dystrophin-deficient brain from a genetic mouse model of Duchenne muscular dystrophy. |
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