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Improved brain and muscle mitochondrial respiration with CoQ. An in vivo study by 31 P‐MR spectroscopy in patients with mitochondrial cytopathies
Author(s) -
Barbiroli Bruno,
Iotti Stefano,
Lodi Raffaele
Publication year - 1999
Publication title -
biofactors
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.204
H-Index - 94
eISSN - 1872-8081
pISSN - 0951-6433
DOI - 10.1002/biof.5520090221
Subject(s) - mitochondrion , in vivo , skeletal muscle , respiration , oxidative phosphorylation , biology , atp synthase , mitochondrial myopathy , medicine , endocrinology , biochemistry , chemistry , mitochondrial dna , enzyme , anatomy , gene , genetics
We used in vivo phosphorus magnetic resonance spectroscopy ( 31 P‐MRS) to study the effect of CoQ 10 on the efficiency of brain and skeletal muscle mitochondrial respiration in ten patients with mitochondrial cytopathies. Before CoQ, brain [PCr] was remarkably lower in patients than in controls, while [Pi] and [ADP] were higher. Brain cytosolic free [Mg 2+ ] and Δ G of ATP hydrolysis were also abnormal in all patients. MRS also revealed abnormal mitochondrial function in the skeletal muscles of all patients, as shown by a decreased rate of PCr recovery from exercise. After six‐months of treatment with CoQ (150 mg/day), all brain MRS‐measurable variables as well as the rate of muscle mitochondrial respiration were remarkably improved in all patients. These in vivo findings show that treatment with CoQ in patients with mitochondrial cytopathies improves mitochondrial respiration in both brain and skeletal muscles, and are consistent with Lenaz's view that increased CoQ concentration in the mitochondrial membrane increases the efficiency of oxidative phosphorylation independently of enzyme deficit.