Role of cardiolipin in mitochondrial signaling pathways

J Dudek - Frontiers in cell and developmental biology, 2017 - frontiersin.org
The phospholipid cardiolipin (CL) is an essential constituent of mitochondrial membranes
and plays a role in many mitochondrial processes, including respiration and energy …

Making heads or tails of phospholipids in mitochondria

C Osman, DR Voelker, T Langer - Journal of Cell Biology, 2011 - rupress.org
Mitochondria are dynamic organelles whose functional integrity requires a coordinated
supply of proteins and phospholipids. Defined functions of specific phospholipids, like the …

Modeling the mitochondrial cardiomyopathy of Barth syndrome with induced pluripotent stem cell and heart-on-chip technologies

G Wang, ML McCain, L Yang, A He, FS Pasqualini… - Nature medicine, 2014 - nature.com
Study of monogenic mitochondrial cardiomyopathies may yield insights into mitochondrial
roles in cardiac development and disease. Here, we combined patient-derived and …

Mitochondrial dysfunction-related lipid changes occur in nonalcoholic fatty liver disease progression

KY Peng, MJ Watt, S Rensen, JW Greve, K Huynh… - Journal of lipid …, 2018 - ASBMB
Nonalcoholic fatty liver disease (NAFLD) comprises fat-accumulating conditions within
hepatocytes that can cause severe liver damage and metabolic comorbidities. Studies …

Barth syndrome

SLN Clarke, A Bowron, IL Gonzalez, SJ Groves… - Orphanet journal of rare …, 2013 - Springer
First described in 1983, Barth syndrome (BTHS) is widely regarded as a rare X-linked
genetic disease characterised by cardiomyopathy (CM), skeletal myopathy, growth delay …

Biosynthesis, remodeling and turnover of mitochondrial cardiolipin

M Schlame, ML Greenberg - … et Biophysica Acta (BBA)-Molecular and Cell …, 2017 - Elsevier
Among mitochondrial lipids, cardiolipin occupies a unique place. It is the only phospholipid
that is specific to mitochondria and although it is merely a minor component, accounting for …

The complexity of cardiolipin in health and disease

SM Claypool, CM Koehler - Trends in biochemical sciences, 2012 - cell.com
Cardiolipin, the signature phospholipid of mitochondria, is a lipid dimer that is important for a
diverse range of mitochondrial activities beyond the process of ATP production. Thus not …

Manganese superoxide dismutase: guardian of the powerhouse

AK Holley, V Bakthavatchalu, JM Velez-Roman… - International journal of …, 2011 - mdpi.com
The mitochondrion is vital for many metabolic pathways in the cell, contributing all or
important constituent enzymes for diverse functions such as β-oxidation of fatty acids, the …

The alpha keto amide moiety as a privileged motif in medicinal chemistry: current insights and emerging opportunities

M Robello, E Barresi, E Baglini, S Salerno… - Journal of Medicinal …, 2021 - ACS Publications
Over the years, researchers in drug discovery have taken advantage of the use of privileged
structures to design innovative hit/lead molecules. The α-ketoamide motif is found in many …

Cardiac and skeletal muscle defects in a mouse model of human Barth syndrome

D Acehan, F Vaz, RH Houtkooper, J James… - Journal of biological …, 2011 - ASBMB
Barth syndrome is an X-linked genetic disorder caused by mutations in the tafazzin (taz)
gene and characterized by dilated cardiomyopathy, exercise intolerance, chronic fatigue …