On the Pathogenesis of Mitochondrial Myopathies. An Experimental Study

Overview

Journal Acta Neuropathol

Specialty Neurology

Date 1979 May 15

PMID 223362

Citations 7

Authors

V Sahgal

V Subramani

R Hughes

A Shah

H Singh

Affiliations

Soon will be listed here.

Abstract

The intra-arterial injection of 2-4 dinitrophenol, an uncoupler of oxidative phosphorylation, resulted in the production of ragged red fibers. The ultrastructure of these fibers showed intramitochondrial paracrystalline inclusions, laminar and fingerprint bodies. Antimycin A and oligomycin injection (which inhibit mitochondrial respiration) only caused swelling and disruption of the mitochondria. An increase in muscle lactic acid, decrease in ATP, glycogen and phosphocreatine was observed after the injection of all these agents. This indicates that lactic acidosis has no significant role in the pathogenesis of mitochondrial pathology. It is concluded that mitochondrial changes are a morphological expression of uncoupled but intact mitochondrial respiration.

Citing Articles

Ultrastructural study of the childhood mitochondrial myopathic syndrome associated with lactic acidosis.

Kobayashi Y, Miyabayashi S, Takada G, Narisawa K, Tada K, Yamamoto T Eur J Pediatr. 1982; 139(1):25-30.

PMID: 7173254 DOI: 10.1007/BF00442074.

Ultrastructural changes in fibroblast mitochondria of a patient with HHH-syndrome.

Metoki K, Hommes F, Dyken P, Kelloes C, Trefz J J Inherit Metab Dis. 1984; 7(4):147-50.

PMID: 6441860 DOI: 10.1007/BF01805595.

Ultrastructure of muscle spindle in congenital myotonic dystrophy. A study of preterm infant muscle spindles.

Sahgal V, Sahgal S, Bernes S, Subramani V Acta Neuropathol. 1983; 61(3-4):207-13.

PMID: 6228107 DOI: 10.1007/BF00691987.

Effects of oral supplementation with stable strontium.

Skoryna S Can Med Assoc J. 1981; 125(7):703-12.

PMID: 6120036 PMC: 1862446.

Experimentally induced defects of mitochondrial metabolism in rat skeletal muscle. Biological effects of the mitochondrial uncoupling agent 2,4-dinitrophenol.

Byrne E, Hayes D, Shoubridge E, Morgan-Hughes J, Clark J Biochem J. 1985; 229(1):101-8.

PMID: 4038252 PMC: 1145155. DOI: 10.1042/bj2290101.

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