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To the Editor:
We read with interest the reports by Hirano et al.1 and Lara et al.2 describing allogeneic stem cell transplantation and repeated platelet transfusions respectively as potential therapies for the mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) syndrome.
However, we were surprised to read the assertion by Chinnery and Vissing in the accompanying editorial that successful treatment of MNGIE might represent the first cure for a mitochondrial disorder.3 We would like to remind your wider readership that disorders of biosynthesis of coenzyme Q10 (Co-Q10), the mobile electron carrier responsible for transferring electrons from complexes I and II to complex III of the mitochondrial respiratory chain, were the first treatable mitochondrial myopathies to be reported almost 20 years ago.4
Diagnosis is confirmed by assay of Co-Q10 in skeletal muscle or peripheral blood mononuclear cells.5 The clinical spectrum of primary Co-Q10 deficiency includes myopathy with recurrent rhabdomyolysis, ataxia, Leigh syndrome, and a multisystem disorder in which encephalomyopathy and nephropathy are prominent clinical features.6
Recently, the first genetic defect of Co-Q10 biosynthesis was reported, a mutation in …
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