Direct measurement of high-energy phosphate compounds in patients with neuromuscular disease.
Several neuromuscular disorders are associated with reductions in intramuscular adenosine triphosphate (ATP) and/or phosphocreatine (PCr). These alterations have been primarily characterized using (31)P-magnetic resonance spectroscopy ((31)P-MRS). We prospectively measured total creatine, PCr, and ATP in muscle biopsies from 81 patients: normal controls (n = 33), mitochondrial cytopathy (n = 8), neuropathic (n = 3), dystrophy/congenital myopathies (n = 7), inflammatory myopathy (n = 12), and miscellaneous myopathies (n = 18) using direct biochemical analysis. Intramuscular concentrations of PCr and ATP were lower for the dystrophy/congenital myopathy, inflammatory myopathy, and mitochondrial disease patients with ragged red fiber (RRF) as compared with normal controls (P < 0.05). Total creatine was lower for the dystrophy/congenital myopathy group as compared with the normal control group (P < 0.05). These values compare favorably to results from other studies using (31)P-MRS and provide external validation for the values obtained using that method. Given the reductions in high-energy phosphate compounds in these patients, there is the potential for therapeutic intervention with creatine monohydrate supplementation.
Tarnopolsky
M A
MA
Department of Neurology/Neurological Rehabilitation, Rm. 4U4, McMaster University Medical Center, 1200 Main St. W., Hamilton, Ontario, Canada, L8N 3Z5.
Parise
G
G
D000255:Adenosine Triphosphate / Q000032:analysis*; D000328:Adult; D000368:Aged; D001706:Biopsy; D003401:Creatine / Q000032:analysis*; D005260:Female; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008875:Middle Aged; D009132:Muscles / Q000737:chemistry*; D009468:Neuromuscular Diseases / Q000378:metabolism*; D010725:Phosphocreatine / Q000032:analysis*
D010725:Phosphocreatine; D000255:Adenosine Triphosphate; D003401:Creatine