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Article

Monolysocardiolipin in cultured fibroblasts is a sensitive and specific marker for Barth Syndrome.

Journal of lipid research - 1 Oct 2006

van Werkhoven Michiel Adriaan, Thorburn David Ross, Gedeon Agi Kyra, Pitt James Jonathon

Abstract excerpt

Barth Syndrome (BTHS) is an X-linked recessive disorder that results in abnormal metabolism of the mitochondrial phospholipid cardiolipin (CL). CLs are decreased and monolysocardiolipins (MLCLs), intermediates in CL metabolism, are increased in a variety of tissues. Measurement of decreased CL levels in skin fibroblasts has previously been proposed as a diagnostic test for BTHS. We investigated whether elevated...

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Source

Identifiers

PMID: 16873891
DOI: 10.1194/jlr.D600024-JLR200

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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