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Oemer 2016b Abstract Mito Xmas Meeting Innsbruck

From Bioblast
The impact of mitochondrial phospholipid remodeling on mitochondrial function.

Link:

Oemer G, Zschocke J, Keller MA (2016)

Event: Mito Xmas Meeting 2016 Innsbruck AT

Mitochondria are highly organized into the outer mitochondrial membrane and the extensively folded cristae structure of the inner mitochondrial membrane (IMM). These membranes are vital for providing the embedding environment of membrane bound complexes such as the respiratory chain and the mitochondrial protein import machinery. Main components of mitochondrial membranes are glycerophospholipids, among which the mitochondria specific cardiolipins are abundant with up to 20% in the IMM.

Cardiolipins represent unique lipids with a glycerol bridged diphosphatidylglycerol backbone that can be substituted with up to four acyl chains. The generated wedge like structure is optimal for facilitating the curved cristae, while the backbone acts as proton trap and buffers pH fluctuations generated in mitochondria. The biosynthesis of cardiolipins is facilitated in a multi-step, multi-compartment manner, initiated from phosphatidic acid in the endoplasmic reticulum and finally forming cardiolipins in the IMM. As the biosynthesis enzymes are unspecific in respect to the acyl side chains of their substrates, a post-biosynthetic side-chain remodeling process is required to generate a mature and functional cardiolipin profile. Patients with impaired cardiolipin remodeling process are for example suffering from the x-linked Barth Syndrome, or the MEGDEL syndrome. In affected individuals the impaired membrane assembly causes mitochondrial dysfunctions resulting in common symptoms such as 3-methylglutaconic aciduria, cardiomyopathy, and muscle weakness.

We recently developed a lipidomics platform that allows us to comprehensively quantify alterations in respective mitochondrial lipid compositions, to connect these data with mitochondrial functional parameters, and consequently to elucidate the largely uncharacterized pathobiochemistry of these diseases.


Labels: MiParea: mt-Membrane, Patients  Pathology: Inherited, Myopathy  Stress:Oxidative stress;RONS, Mitochondrial disease  Organism: Human, Pig, Mouse, Zebrafish, Drosophila, Saccharomyces cerevisiae  Tissue;cell: Heart, Skeletal muscle, Liver, Kidney, Lung;gill, Fibroblast, Macrophage-derived  Preparation: Homogenate 

Regulation: Fatty acid 


Event: A2, Oral  Labelled by author 

Affiliations

Oemer G, Zschocke J, Keller MA
  1. Division Human Genetics, Medical Univ Innsbruck, Austria