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Ultrastructural changes in fibroblast mitochondria of a patient with HHH‐syndrome
Author(s) -
Metoki K.,
Hommes F. A.,
Dyken P.,
Kelloes C.,
Trefz J.
Publication year - 1984
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01805595
Subject(s) - ultrastructure , mitochondrion , fibroblast , electron microscope , inner mitochondrial membrane , electron micrographs , biology , chemistry , pathology , medicine , biochemistry , physics , in vitro , optics
Electron micrographs of fibroblasts of an HHH‐syndrome patient showed abnormal structures, similar, but not identical, to those observed in the liver of such patients. It is suggested that incorporation of a mutated protein into the inner mitochondrial membrane gives rise to a rearrangement of that membrane, resulting in unusual structures.