Difference between revisions of "Balbaisi 2022 BEC"
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|abstract=[[File:BEC.png|25px|link=https://doi.org/10.26124/bec:2022-0005]] https://doi.org/10.26124/bec:2022-0005 | |abstract=[[File:BEC.png|25px|link=https://doi.org/10.26124/bec:2022-0005]] https://doi.org/10.26124/bec:2022-0005 | ||
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 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 transfer system (ETS) and its components, and hence CL is required for efficient mitochondrial bioenergetics. Mutations in CL remodeling enzyme encoded by the ''TAFAZZIN'' gene 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.<br><br> | 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 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 transfer system (ETS) and its components, and hence CL is required for efficient mitochondrial bioenergetics. Mutations in CL remodeling enzyme encoded by the ''TAFAZZIN'' gene 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.<br><br> | ||
|keywords=Barth syndrome, | |keywords=Barth syndrome, ''TAFAZZIN'', cardiolipin, cardiolipin remodeling, 3-MGA, respiratory Complexes<br> | ||
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|editor=Tindle-Solomon Lisa | |editor=Tindle-Solomon Lisa | ||
}} | }} | ||
ORC'''ID''': [[File:ORCID.png|20px|link=https://orcid.org/0000-0002-3748-9976]] Stiban Johnny | ORC'''ID''': [[File:ORCID.png|20px|link=https://orcid.org/0000-0002-3748-9976]] Stiban Johnny | ||
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== Preprint == | |||
::::Β» [[Balbaisi 2022 MitoFit]] | |||
{{Labeling | {{Labeling |
Revision as of 11:17, 12 July 2022
Balbaisi A, Stiban J (2022) Barth syndrome: a genetic ailment with a lipid component and bioenergetic ramifications. https://doi.org/10.26124/bec:2022-0005 |
Β» Bioenerg Commun 2022.5. published online 2022-06-28
Balbaisi Abdallah, Stiban Johnny (BEC 2022.5) Bioenerg Commun
Abstract: https://doi.org/10.26124/bec:2022-0005
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 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 transfer system (ETS) and its components, and hence CL is required for efficient mitochondrial bioenergetics. Mutations in CL remodeling enzyme encoded by the TAFAZZIN gene 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.
β’ Keywords: Barth syndrome, TAFAZZIN, cardiolipin, cardiolipin remodeling, 3-MGA, respiratory Complexes
β’ Bioblast editor: Tindle-Solomon Lisa
Preprint
Labels: MiParea: mt-Membrane
Pathology: Inherited
Organism: Human
BEC