Dynamic organization of mitochondria in human heart and in myocardial disease.
Heart mitochondria, which, depending on their location within cardiomyofibers, are classified as either subsarcolemmal or interfibrillar, are the major sources of the high energy compound, adenosine triphosphate. Physiological differences between these two populations are reflected by differences in the morphology of their cristae, with those of subsarcolemmal mitochondria being mostly lamelliform, and those of interfibrillar mitochondria being mostly tubular. What determines the configuration of cristae, not only in cardiac mitochondria but in mitochondria in general, is unclear. The morphology of cardiac mitochondria, as well as their physiology, is responsive to the exigencies posed by a large variety of pathological situations. Giant cardiac mitochondria make an appearance in certain types of cardiomyopathy and as a result of dietary, pharmacological, and toxicological manipulation; such megamitochondria probably arise by a combination of fusion and true growth. Some of these enlarged organelles occasionally contain a membrane-bound deposit of beta-glycogen. Those giant mitochondria induced by experimental treatment usually can be restored to normal dimensions simply by supplying the missing nutrient or by deleting the noxious substance. In some conditions, such as endurance training and ischemia, the mitochondrial matrices become pale. Dense rods or plates are present in the outer compartment of mitochondria under certain conditions. Biochemical alterations in cardiac mitochondria appear to be important in heart failure. In aging, only interfibrillar mitochondria exhibit such changes, with the subsarcolemmal mitochondria unaffected. In certain heart afflictions, biochemical defects are not accompanied by obvious morphological transformations. Mitochondria clearly play a cardinal role in homeostasis of the heart.
Hoppel
Charles L
CL
Department of Pharmacology and Medicine and Center for Mitochondrial Disease, School of Medicine, Case Western Reserve University, 10900 Euclid Avenue, Cleveland, OH 44106, USA. Charles.Hoppel@case.edu
Tandler
Bernard
B
Fujioka
Hisashi
H
Riva
Alessandro
A
D009202:Cardiomyopathies / Q000378:metabolism* / Q000503:physiopathology; D006801:Humans; D008854:Microscopy, Electron; D008929:Mitochondria, Heart / Q000378:metabolism*; D010085:Oxidative Phosphorylation