Mitochondrial membrane lipids in the regulation of bioenergetic flux

ST Decker, K Funai - Cell metabolism, 2024 - cell.com
Oxidative phosphorylation (OXPHOS) occurs through and across the inner mitochondrial
membrane (IMM). Mitochondrial membranes contain a distinct lipid composition, aided by …

Beyond fission and fusion—Diving into the mysteries of mitochondrial shape

N Preminger, M Schuldiner - Plos Biology, 2024 - journals.plos.org
Mitochondrial shape and network formation have been primarily associated with the well-
established processes of fission and fusion. However, recent research has unveiled an …

Metabolic switch from fatty acid oxidation to glycolysis in knock‐in mouse model of Barth syndrome

A Chowdhury, A Boshnakovska, A Aich… - EMBO molecular …, 2023 - embopress.org
Mitochondria are central for cellular metabolism and energy supply. Barth syndrome (BTHS)
is a severe disorder, due to dysfunction of the mitochondrial cardiolipin acyl transferase …

Barth syndrome cardiomyopathy: an update

J Pang, Y Bao, K Mitchell-Silbaugh, J Veevers, X Fang - Genes, 2022 - mdpi.com
Barth syndrome (BTHS) is an X-linked mitochondrial lipid disorder caused by mutations in
the TAFAZZIN (TAZ) gene, which encodes a mitochondrial acyltransferase/transacylase …

Activation of the integrated stress response rewires cardiac metabolism in Barth syndrome

I Kutschka, E Bertero, C Wasmus, K Xiao… - Basic Research in …, 2023 - Springer
Barth Syndrome (BTHS) is an inherited cardiomyopathy caused by defects in the
mitochondrial transacylase TAFAZZIN (Taz), required for the synthesis of the phospholipid …

Cardiolipin regulates mitochondrial ultrastructure and function in mammalian cells

Z Jiang, T Shen, H Huynh, X Fang, Z Han, K Ouyang - Genes, 2022 - mdpi.com
Cardiolipin (CL) is a unique, tetra-acylated diphosphatidylglycerol lipid that mainly localizes
in the inner mitochondria membrane (IMM) in mammalian cells and plays a central role in …

Ginsenoside Rg3 restores mitochondrial Cardiolipin homeostasis via GRB2 to prevent Parkinson's disease

LFR Qi, S Liu, Q Fang, C Qian, C Peng, Y Liu… - Advanced …, 2024 - Wiley Online Library
Regulating cardiolipin to maintain mitochondrial homeostasis is a promising strategy for
addressing Parkinson's disease (PD). Through a comprehensive screening and validation …

Mitochondrial cardiomyopathy: molecular epidemiology, diagnosis, models, and therapeutic management

J Yang, S Chen, F Duan, X Wang, X Zhang, B Lian… - Cells, 2022 - mdpi.com
Mitochondrial cardiomyopathy (MCM) is characterized by abnormal heart-muscle structure
and function, caused by mutations in the nuclear genome or mitochondrial DNA. The …

Mitochondrial stress induces an HRI-eIF2α pathway protective for cardiomyopathy

S Zhu, A Nguyen, J Pang, J Zhao, Z Chen, Z Liang… - Circulation, 2022 - Am Heart Assoc
Mitochondrial phosphatase protein tyrosine phosphatase mitochondrial 1 (Ptpmt1) cKO
(PKO) mice2 provide a model to investigate in vivo mechanisms of MSR in cardiomyocytes …

DELE1 is protective for mitochondrial cardiomyopathy

H Huynh, S Zhu, S Lee, Y Bao, J Pang… - Journal of molecular and …, 2023 - Elsevier
Mitochondrial dysfunction in heart triggers an integrated stress response (ISR) through
phosphorylation of eIF2α and subsequent ATF4 activation. DAP3 Binding Cell Death …