linoleic-acid and Mitochondrial-Myopathies

linoleic-acid has been researched along with Mitochondrial-Myopathies* in 1 studies

Other Studies

1 other study(ies) available for linoleic-acid and Mitochondrial-Myopathies

Article	Year
Deficiency of tetralinoleoyl-cardiolipin in Barth syndrome. Annals of neurology, 2002, Volume: 51, Issue:5 Barth syndrome is an X-linked cardiac and skeletal mitochondrial myopathy. Barth syndrome may be due to lipid alterations because the product of the mutated gene is homologous to phospholipid acyltransferases. Here we document that a single mitochondrial phospholipid species, tetralinoleoyl-cardiolipin, was lacking in the skeletal muscle (n = 2), right ventricle (n = 2), left ventricle (n = 2), and platelets (n = 6) of 8 children with Barth syndrome. Tetralinoleoyl-cardiolipin is specifically enriched in normal skeletal muscle and the normal heart. These findings support the notion that Barth syndrome is caused by alterations of mitochondrial lipids. Topics: Adolescent; Adult; Aged; Animals; Blood Platelets; Cardiolipins; Cell Line; Child; Child, Preschool; Dogs; Female; Humans; Infant; Linoleic Acid; Male; Mice; Mice, Inbred C3H; Middle Aged; Mitochondria, Heart; Mitochondrial Myopathies; Muscle, Skeletal; Myocardium; Rats; Rats, Sprague-Dawley	2002

Article

Year

Deficiency of tetralinoleoyl-cardiolipin in Barth syndrome.

Annals of neurology, 2002, Volume: 51, Issue:5

Barth syndrome is an X-linked cardiac and skeletal mitochondrial myopathy. Barth syndrome may be due to lipid alterations because the product of the mutated gene is homologous to phospholipid acyltransferases. Here we document that a single mitochondrial phospholipid species, tetralinoleoyl-cardiolipin, was lacking in the skeletal muscle (n = 2), right ventricle (n = 2), left ventricle (n = 2), and platelets (n = 6) of 8 children with Barth syndrome. Tetralinoleoyl-cardiolipin is specifically enriched in normal skeletal muscle and the normal heart. These findings support the notion that Barth syndrome is caused by alterations of mitochondrial lipids.

Topics: Adolescent; Adult; Aged; Animals; Blood Platelets; Cardiolipins; Cell Line; Child; Child, Preschool; Dogs; Female; Humans; Infant; Linoleic Acid; Male; Mice; Mice, Inbred C3H; Middle Aged; Mitochondria, Heart; Mitochondrial Myopathies; Muscle, Skeletal; Myocardium; Rats; Rats, Sprague-Dawley

2002