The Function of Tafazzin, a Mitochondrial Phospholipid-Lysophospholipid Acyltransferase.

Abstract:

:Tafazzin is a mitochondrial enzyme that exchanges fatty acids between phospholipids by phospholipid-lysophospholipid transacylation. The reaction alters the molecular species composition and, as a result, the physical properties of lipids. In vivo, the most important substrate of tafazzin is the mitochondria-specific lipid cardiolipin. Tafazzin mutations cause the human disease Barth syndrome, which presents with cardiomyopathy, skeletal muscle weakness, fatigue, and other symptoms, probably all related to mitochondrial dysfunction. The reason why mitochondria require tafazzin is still not known, but recent evidence suggests that tafazzin may lower the energy cost associated with protein crowding in the inner mitochondrial membrane.

journal_name

J Mol Biol

authors

Schlame M,Xu Y

doi

10.1016/j.jmb.2020.03.026

subject

Has Abstract

pub_date

2020-08-21 00:00:00

pages

5043-5051

issue

18

eissn

0022-2836

issn

1089-8638

pii

S0022-2836(20)30259-X

journal_volume

432

pub_type

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