Work

A Case of Severe Hypermetabolism of Nonthyroid Origin With a Defect in the Maintenance of Mitochondrial Respiratory Control: a Correlated Clinical, Biochemical, and Morphological Study

Year: 1962

Type: article

Abstract: Symptomatic hypermetabolism is often encoun-tered in clinical medicine and is mostly due to thy-roid diseases. In 1959, Ernster, Ikkos and Luft (1) presented preliminary biochemical studies on isolated skeletal muscle mitochondria from a patient with severe hypermetabolism of unknown origin. A defect of the biochemical function of these mitochondria was found, and it was sug-gested that this defect might be responsible for the hypermetabolic state of the patient. It is now well established that mitochondria are the site of cellular respiration as well as of the process called oxidative phosphorylation by which energy liberated in respiration is conserved in the form of ATP 1 (2). Normally the respiration of isolated mitochondria is obligatorily coupled to the formation of ATP from ADP and inorganic phosphate, and thus cannot proceed at any ap-preciable rate if either of the latter components is absent from the incubating medium (3). This phenomenon, usually termed respiratory control, is generally considered to be a reflection of the ca-pacity of the living cell to regulate its respiration according to its actual demand of energy. It was found that in the present patient this ability of the isolated skeletal muscle mitochondria was severely

Source: Journal of Clinical Investigation

DOI: 10.1172/jci104637

Authors: Rolf Luft, Denis Ikkos, Genaro Palmieri, Lars Ernster, Björn Afzelius

Institutions: Hospital Privado, Evangelismos Hospital, Karolinska University Hospital, Stockholm University

Language: English

Topic: Mitochondrial Function and Pathology

Subfield: Molecular Biology

Field: Biochemistry, Genetics and Molecular Biology

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: green

Funder: Medical Research Council