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NEUROLOGY 1987;37:1184
© 1987 American Academy of Neurology

Exercise-induced recurrent myoglobinuria

Defective activity of inner carnitine palmitoyltransferase in muscle mitochondria of two patients

Carlo P. Trevisan, MD, Grazia Isaya, MD and Corrado Angelini, MD

Regional Center for Neuromuscular Diseases, Neurological Clinic, University of Padova, Padova, Italy.

In carnitine palmitoyltransferase (CPT) deficiency, it is not known whether the outer (CPT-I) and the inner (CPT-II) mitochondrial activities are equally altered. By two different assays, we found that CPT activity in fresh intact mitochondria of two patients was normal or increased, indicating an active outer CPT. In controls and in one of the two patients, the isotope-exchange assay was also evaluated after disruption of mitochondria by sonication: in controls the activity almost doubled because of the contribution of inner CPT to the assay, but in the patients it did not increase, indicating absence of the CPT-II activity. After further disruption of mitochondria by freezing and all-glass homogenization, CPT activity in patients decreased to 36% and 10% of control. These data suggest that CPT deficiency was limited to the inner mitochondrial activity. The alteration could be explained by mutation of the membrane factor that determines in situ differences between CPT-I and II.

Address correspondence to Dr. Trevisan, Clinica Neurologica, Università di Padova, Via Giustiniani 5, I-35128, Padova, Italy.

Supported by "Legato Dino Ferrari" and Italy C. N. R. grants.

Received March 18, 1986. Accepted for publication in final form November 17, 1986.






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