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Balbaisi 2022 MitoFit

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Revision as of 11:44, 9 March 2022 by Tindle Lisa (talk | contribs) (Created page with "{{MitoFit page name}} {{Publication |title=Balbaisi A, Stiban J (2022) Barth Syndrome: A Genetic Ailment with a Lipid Component and Bioenergetic Ramifications. MitoFit Prepri...")
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Balbaisi 2022 MitoFit


Publications in the MiPMap
Balbaisi A, Stiban J (2022) Barth Syndrome: A Genetic Ailment with a Lipid Component and Bioenergetic Ramifications. MitoFit Preprints 2022.3. doi:10.26124/mitofit:2022-0003

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Barth Syndrome: A Genetic Ailment with a Lipid Component and Bioenergetic Ramifications

Balbaisi Abdallah, Stiban Johnny (2022-03-09) MitoFit Prep

Abstract: In eukaryotes membranes are structural components that are necessary for compartmentalization of function. Membranes consist of a lipid bilayer with a multitude of proteins on or in this sandwich. Nevertheless, membranes are not solely structural in function but also, they serve as basis for cellular signaling and metabolism. Membranes vary with respect to their lipid composition, protein:lipid ratio, thickness, carbohydrate content, etc., and hence their functions are not necessarily identical in the different compartments. In the mitochondrial inner membrane (mtIM), as in its bacterial ancestor, a special phospholipid is present. Cardiolipin (CL) is a phospholipid consisting of four hydrophobic tails. It is essential for the assembly of the electron transport system (ETS) and its components, and hence CL is required for efficient mitochondrial bioenergetics. Mutations in CL remodeling enzyme encoded by the tafazzin gene (TAZ) are associated with a syndrome first identified by Dutch scientist Peter Barth, hence the name Barth Syndrome. Here, we review recent research on this devastating syndrome focusing on CL biosynthesis and remodeling and relationship between the phospholipid component and mitochondrial bioenergetics. We further by exploring management and possible future techniques in the treatment of this syndrome.

β€’ Keywords: Barth syndrome, tafazzin, cardiolipin, cardiolipin remodeling, 3-MGA, respiratory complexes β€’ Bioblast editor: Tindle-Solomon L

ORCID: ORCID.png Stiban Johnny


Labels: MiParea: Respiration  Pathology: Other 

Organism: Rat  Tissue;cell: Liver, Blood cells  Preparation: Permeabilized cells, Homogenate  Enzyme: Marker enzyme 

Coupling state: LEAK, ROUTINE, OXPHOS, ET  Pathway: N, S, NS, Other combinations, ROX  HRR: Oxygraph-2k