MOLSIDOMINE, a NO donor
Molsidomine NO donor.
(Neurobiology of Disease, 2009; 36 (2): 252-258) MALDI reveals membrane lipid profile reversion in MDX mice
Farida Benabdellah, Hua Yu, Alain Brunelle, Olivier Laprévote, Sabine De La Porte - France
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.
(Neurobiology of Disease, 2009; 36 (2): 252-258) MALDI reveals membrane lipid profile reversion in MDX mice
Farida Benabdellah, Hua Yu, Alain Brunelle, Olivier Laprévote, Sabine De La Porte - France
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.
Replies to This Discussion
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Permalink Reply by Luis Cordero on
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I received this msg from this research group
"Dear Sir
Thank you for your interest in our work.
I don't think that molsidomine is planned to be shortly used by AFM (Association Française contre les Myopathies) or MDA (Muscular Dystrophy
Association). Both associations privileged cell and gene therapies more than pharmacological therapy.
Nevertheless: the doctor which take care of your father can prescribe him Corvasal
Corvasal is frequently used in human. I can't give you any indication concerning the dose to used in human, I am not doctor in medecine.
In mice, we recently performed some tests with lower doses that the 10mg/kg/day used in the publication you known. We tried 5 , 2 and 1 mg/kg/day and the
results were good (the CK level was clearly reduced ) ".
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Hello
i am very interested by this , as i 'm french i can contacted them.
can you give me the e mail address and the weblink of their article
best
françoise
Luis Cordero said:I received this msg from this research group
"Dear Sir
Thank you for your interest in our work.
I don't think that molsidomine is planned to be shortly used by AFM (Association Française contre les Myopathies) or MDA (Muscular Dystrophy
Association). Both associations privileged cell and gene therapies more than pharmacological therapy.
Nevertheless: the doctor which take care of your father can prescribe him Corvasal
Corvasal is frequently used in human. I can't give you any indication concerning the dose to used in human, I am not doctor in medecine.
In mice, we recently performed some tests with lower doses that the 10mg/kg/day used in the publication you known. We tried 5 , 2 and 1 mg/kg/day and the
results were good (the CK level was clearly reduced ) ".
-
-
Françoise
This is the research contact
Sabine De La Porte
sporte@nbcm.cnrs-gif.fr
best regards
Luis
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Permalink Reply by Traci Strafuss on
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They state that introducing NO could lead to an increase in utrophin and a regression of the dystrophic phenotype. I do not understand why there are no trials of this drug or another NO donor, such as Isosorbide.
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hi
i don't understand either. has somebody the whole article? , i would like to read it. after i will get in contact with the research team
best
françoise
Traci Strafuss said:They state that introducing NO could lead to an increase in utrophin and a regression of the dystrophic phenotype. I do not understand why there are no trials of this drug or another NO donor, such as Isosorbide.
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