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Loss of protein association causes cardiolipin degradation in Barth syndrome

Loss of protein association causes cardiolipin degradation in Barth syndrome

https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_4955704

Loss of protein association causes cardiolipin degradation in Barth syndrome

About this item

Full title

Loss of protein association causes cardiolipin degradation in Barth syndrome

Publisher

New York: Nature Publishing Group US

Journal title

Nature chemical biology, 2016-08, Vol.12 (8), p.641-647

Language

English

Formats

Publication information

Publisher

New York: Nature Publishing Group US

More information

Scope and Contents

Contents

Monolyso-cardiolipin (MLCL) accumulates in individuals with Barth syndrome, but this can be mitigated by stabilization of cardiolipin from metabolism to MLCL via assembly into supercomplexes of oxidative phosphorylation proteins. This process was found to be defective in subjects with Barth syndrome.
Cardiolipin is a specific mitochondrial phosp...

Alternative Titles

Full title

Loss of protein association causes cardiolipin degradation in Barth syndrome

Identifiers

Primary Identifiers

Record Identifier

TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_4955704

Permalink

https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_4955704

Other Identifiers

ISSN

1552-4450

E-ISSN

1552-4469

DOI

10.1038/nchembio.2113

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