Cardiolipin Remodeling Defects Impair Mitochondrial Architecture ...

Cardiomyopathy is a major clinical feature in Barth syndrome (BTHS), an X-linked mitochondrial lipid disorder caused by mutations in Tafazzin (TAZ).

Background: Cardiomyopathy is a major clinical feature in Barth syndrome (BTHS), an X-linked mitochondrial lipid disorder caused by mutations in ...

Cardiolipin remodeling by ALCAT1 links mitochondrial dysfunction ...

Cardiolipin (CL) is a mitochondrial signature phospholipid that is required for membrane structure, respiration, dynamics, and mitophagy. Oxidative damage of CL ...

Cardiolipin Remodeling Defects Impair Mitochondrial Architecture ...

Request PDF | Cardiolipin Remodeling Defects Impair Mitochondrial Architecture and Function in a Murine Model of Barth Syndrome Cardiomyopathy | Background ...

Remodeling abnormalities in mitochondrial trifunctional protein ...

Mitochondrial bioenergetics and cardiolipin remodeling abnormalities in mitochondrial ... affect protein tertiary structure (C bottom left).

Cardiolipin remodeling in Barth syndrome and other hereditary ...

Cardiolipin is a phospholipid found in the inner mitochondrial membrane and involved in ATP production and metabolism. •. Defects in cardiolipin biosynthesis ...

Defective Mitochondrial Cardiolipin Remodeling Dampens HIF-1α ...

ROS-mediated NF-κB activation is impaired in cardiolipin ... Accordingly, defects in mitochondrial functions are frequently linked to human ...

Cardiolipin Remodeling Defects Impair Mitochondrial Architecture ...

Cardiolipin Remodeling Defects Impair Mitochondrial Architecture and Function in a Murine Model of Barth Syndrome Cardiomyopathy. Siting Zhu, Ze'e Chen ...

Metabolic Alterations Caused by Defective Cardiolipin Remodeling ...

Defects in the biosynthesis and remodeling of CL have a strong impact on mitochondrial function and particularly affect tissues with a high energetic ...

Mitochondrial dysfunction in metabolic and cardiovascular diseases ...

Different reports indicate that the cardiolipin acyl chain composition affect mitochondrial ... Defective mitochondrial cardiolipin remodeling dampens hif-1α ...

[PDF] Role of cardiolipin alterations in mitochondrial dysfunction and ...

Emerging roles of cardiolipin remodeling in mitochondrial dysfunction ... Altered membrane composition and structure and impaired complex I/III ...

Cardiolipin Regulates Mitochondrial Ultrastructure and Function in ...

Cardiolipin Remodeling Defects Impair Mitochondrial Architecture and Function in a Murine Model of Barth Syndrome Cardiomyopathy. Circ ...

Role of Cardiolipin in Mitochondrial Signaling Pathways - Frontiers

Loss of prohibitin membrane scaffolds impairs mitochondrial architecture and leads to tau hyperphosphorylation and neurodegeneration. ... Biosynthesis, remodeling ...

Cardiolipin, Mitochondria, and Neurological Disease - Cell Press

Aberrant CL content, structure, and localization are linked to impaired neurogenesis and neuronal dysfunction, contributing to aging and the pathogenesis of ...

Cardiolipin remodeling by TAZ/tafazzin is selectively required for the ...

Consistent with a key role of mitophagy in mitochondria quality control, TAZ deficiency in MEFs also led to impaired oxidative phosphorylation and severe ...

Barth syndrome: A life-threatening disorder caused by abnormal ...

BTHS is caused by mutations in the gene tafazzin (TAZ), resulting in defective remodeling of cardiolipin (CL), the signature phospholipid of the mitochondrial ...

(PDF) Metabolic Alterations Caused by Defective Cardiolipin ...

... remodeling, changes in mitochondrial structure or function, and alterations in mitochondrial. calcium signaling diminish mitochondrial energy ...

Barth syndrome cardiomyopathy: targeting the mitochondria with ...

... cardiolipin remodeling, it is critical to maintaining mitochondrial inner membrane structure and function. Tafazzin is encoded by the TAZ ...

Tafazzin deficiency impairs CoA-dependent oxidative metabolism in ...

... cardiolipin defects and mitochondrial ... Discussion. This study sought to elucidate how impaired cardiolipin remodeling impacts cardiac mitochondrial ...

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