MALDI reveals membrane lipid profile reversion in MDX mice

• Published in: Neurobiology of disease Vol. 36; no. 2; pp. 252 - 258
• Main Authors: Benabdellah, Farida, Yu, Hua, Brunelle, Alain, Laprévote, Olivier, De La Porte, Sabine
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.11.2009; Elsevier
• Subjects: Animals; Chemical Sciences; Duchenne muscular dystrophy; In situ profiling; Life Sciences; MALDI-MS; mdx; Membrane Lipids - analysis; Membrane Lipids - chemistry; Membrane Lipids - metabolism; Mice; Mice, Inbred C57BL; Mice, Inbred mdx; Molsidomine; Muscle, Skeletal - chemistry; Muscle, Skeletal - metabolism; Muscular Dystrophy, Duchenne - metabolism; Neurology; Neurons and Cognition; NO donor; Organic chemistry; Phosphatidylcholines - analysis; Phosphatidylcholines - chemistry; Regeneration - physiology; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization - methods; mdx; MALDI-MS; Molsidomine; Duchenne muscular dystrophy; In situ profiling; NO donor
• ISSN: 0969-9961; 1095-953X
• DOI: 10.1016/j.nbd.2009.07.013

Abstract Duchenne muscular dystrophy (DMD), the most common and severe X-linked myopathy, is characterized by the lack of dystrophin, a sub-sarcolemmal protein necessary for normal muscle functions. In a previous study of the lipid content of skeletal muscles of dystrophic ( mdx ) mice, the animal model for DMD, by in situ Matrix-Assisted Laser Desorption-Ionization Mass Spectrometry (MALDI-MS), an inversion of the phosphatidylcholine PC34:2/PC34:1 ion peaks intensity ratio was observed between destructured (abnormal fiber morphology) and structured (normal fiber morphology). A possible treatment for this dramatic disease is to introduce an exogenous nitric oxide (NO) donor into the organism, leading to an increase of utrophin and a regression of the dystrophic phenotype. In the present work, after confirmation by tandem mass spectrometry of the structure of these two phospholipids, their intensity ratio inversion was used to evidence a restoration of membrane lipid composition very similar to those of wild-type mice after the treatment of mdx mice with molsidomine, a NO donor. This was associated with the observation by immunohistology of an increase of the regeneration process in the mice.