title: Mitochondrial abnormalities in choroid plexus of Leigh disease.
abstract: Morphological study of the choroid plexuses in three patients with Leigh disease revealed a marked increase in the number of mitochondria in almost all of the choroidal epithelial cells. This finding is considered the morphological expression of a biochemical defect in the mitochondrial metabolism underlying Leigh disease, and the probable explanation for increased CSF levels of lactate and pyruvate in this disease.
authors.affiliation: Department of Pathology, Niigata University, Japan.
authors.forename: E
authors.initials: E
authors.lastname: Ohama
authors.forename: F
authors.initials: F
authors.lastname: Ikuta
authors.forename: N
authors.initials: N
authors.lastname: Nakamura
mesh_terms: D001928:Brain Diseases, Metabolic / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D002831:Choroid Plexus / Q000648:ultrastructure*; D005260:Female; D006801:Humans; D007223:Infant; D007773:Lactates / Q000097:blood; D019344:Lactic Acid; D007888:Leigh Disease / Q000097:blood / Q000473:pathology*; D008297:Male; D008854:Microscopy, Electron; D008928:Mitochondria / Q000648:ultrastructure*; D011773:Pyruvates / Q000097:blood; D019289:Pyruvic Acid
chemical_list: D007773:Lactates; D011773:Pyruvates; D019344:Lactic Acid; D019289:Pyruvic Acid