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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
5
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pubmed:dateCreated |
2002-7-11
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pubmed:abstractText |
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.
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pubmed:grant |
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
May
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pubmed:issn |
0364-5134
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:volume |
51
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
634-7
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pubmed:dateRevised |
2007-11-14
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pubmed:meshHeading |
pubmed-meshheading:12112112-Adolescent,
pubmed-meshheading:12112112-Adult,
pubmed-meshheading:12112112-Aged,
pubmed-meshheading:12112112-Animals,
pubmed-meshheading:12112112-Blood Platelets,
pubmed-meshheading:12112112-Cardiolipins,
pubmed-meshheading:12112112-Cell Line,
pubmed-meshheading:12112112-Child,
pubmed-meshheading:12112112-Child, Preschool,
pubmed-meshheading:12112112-Dogs,
pubmed-meshheading:12112112-Female,
pubmed-meshheading:12112112-Humans,
pubmed-meshheading:12112112-Infant,
pubmed-meshheading:12112112-Linoleic Acid,
pubmed-meshheading:12112112-Male,
pubmed-meshheading:12112112-Mice,
pubmed-meshheading:12112112-Mice, Inbred C3H,
pubmed-meshheading:12112112-Middle Aged,
pubmed-meshheading:12112112-Mitochondria, Heart,
pubmed-meshheading:12112112-Mitochondrial Myopathies,
pubmed-meshheading:12112112-Muscle, Skeletal,
pubmed-meshheading:12112112-Myocardium,
pubmed-meshheading:12112112-Rats,
pubmed-meshheading:12112112-Rats, Sprague-Dawley
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pubmed:year |
2002
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pubmed:articleTitle |
Deficiency of tetralinoleoyl-cardiolipin in Barth syndrome.
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pubmed:affiliation |
Department of Anesthesiology, Hospital for Special Surgery, New York, NY, USA. schlame@hotmail.com
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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