Format

Send to

Choose Destination
See comment in PubMed Commons below
J Bioenerg Biomembr. 2016 Apr;48(2):113-23. doi: 10.1007/s10863-014-9591-7. Epub 2014 Nov 29.

Cardiolipin remodeling: a regulatory hub for modulating cardiolipin metabolism and function.

Author information

1
Department of Biological Sciences, Wayne State University, Detroit, 5047 Gullen Mall, Michigan, 48202, MI, USA.
2
Department of Biological Sciences, Wayne State University, Detroit, 5047 Gullen Mall, Michigan, 48202, MI, USA. mgreenberg@wayne.edu.

Abstract

Cardiolipin (CL), the signature phospholipid of mitochondria, is involved in a plethora of cellular processes and is crucial for mitochondrial function and architecture. The de novo synthesis of CL in the mitochondria is followed by a unique remodeling process, in which CL undergoes cycles of deacylation and reacylation. Specific fatty acyl composition is acquired during this process, and remodeled CL contains predominantly unsaturated fatty acids. The importance of CL remodeling is underscored by the life-threatening genetic disorder Barth syndrome (BTHS), caused by mutations in tafazzin, which reacylates monolysocardiolipin (MLCL) generated from the deacylation of CL. Just as CL-deficient yeast mutants have been instrumental in elucidating functions of this lipid, the recently characterized CL-phospholipase mutant cld1Δ and the tafazzin mutant taz1Δ are powerful tools to understand the functions of CL remodeling. In this review, we discuss recent advances in understanding the role of CL in mitochondria with specific focus on the enigmatic functions of CL remodeling.

KEYWORDS:

Barth syndrome; Bioenergetics; Cardiolipin; Cardiolipin remodeling; Mitochondria; Phospholipase; Tafazzin

PMID:
25432572
PMCID:
PMC4449329
DOI:
10.1007/s10863-014-9591-7
[Indexed for MEDLINE]
Free PMC Article
PubMed Commons home

PubMed Commons

0 comments

    Supplemental Content

    Full text links

    Icon for Springer Icon for PubMed Central
    Loading ...
    Support Center