We studied skeletal muscles from eight chronic progressive external ophthalmoplegia patients with ragged-red fibers (group A), five CPEO patients without ragged-red fibers (group B), and five controls. The EM morphometric fraction of structurally abnormal mitochondria was increased in group A, and there was a similarly increased fraction of normal-appearing mitochondria in group B. State 3 respiration and uncoupled respiration were severely decreased in both groups. The morphometric mitochondrial fraction and respiratory functions were inversely related in all three groups. The cytochrome content in group A was normal; cytochromes b and cc1 were decreased in group B. These studies point to a central role of mitochondrial dysfunction in all types of CPEO, but the basic abnormalities remain to be elucidated.

Chronic progressive external ophthalmoplegia (CPEO): clinical, morphologic, and biochemical studies / H. Mitsumoto, J. R. Aprille, S. H. Wray, R. Nemni, W. G. Bradley. - In: NEUROLOGY. - ISSN 0028-3878. - 33:4(1983 Apr), pp. 452-61-461.

Chronic progressive external ophthalmoplegia (CPEO): clinical, morphologic, and biochemical studies

R. Nemni
Penultimo
;
1983-04

Abstract

We studied skeletal muscles from eight chronic progressive external ophthalmoplegia patients with ragged-red fibers (group A), five CPEO patients without ragged-red fibers (group B), and five controls. The EM morphometric fraction of structurally abnormal mitochondria was increased in group A, and there was a similarly increased fraction of normal-appearing mitochondria in group B. State 3 respiration and uncoupled respiration were severely decreased in both groups. The morphometric mitochondrial fraction and respiratory functions were inversely related in all three groups. The cytochrome content in group A was normal; cytochromes b and cc1 were decreased in group B. These studies point to a central role of mitochondrial dysfunction in all types of CPEO, but the basic abnormalities remain to be elucidated.
Cytochrome c Group; Mitochondria, Muscle; Humans; Adult; Muscles; Cytochrome b Group; Middle Aged; Child; Muscle Proteins; Ophthalmoplegia; Male; Female
Settore MED/26 - Neurologia
Settore MED/30 - Malattie Apparato Visivo
Settore BIO/12 - Biochimica Clinica e Biologia Molecolare Clinica
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/182895
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