In Barth Syndrome cardiolipins are decreased. A ratio of monolysocardiolipin to cardiolipin in cultured cells can aid in the diagnosis.

Location of cardiolipin formation: inner mitochondrial membrane


Method of analysis: HPLC-MS



(1) concentration of nonolysocardiolipin in cultured fibroblasts

(2) concentration of cardiolipin in cultured fibroblasts


ratio =

= (monolysocardiolipin) / (cardiolopin)



The normal ratio is < 0.23.

In Barth syndrome the ratio is > 0.40 (often > 5)

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