Age‐Associated Dysregulation of Postsynaptic Mitochondria Perturbs Reinnervation Kinetics
We show that aging results in postsynaptic mitochondrial loss at the neuromuscular junction and impaired muscle reinnervation. In vivo CRISPR knockout of mitochondrial genes, CHCHD10 and CHCHD2, in young muscles led to fragmented endplates, aberrant innervation, and impaired transcriptional maturation of sub‐synaptic myonuclei during regeneration ...
Steve D. Guzman +8 more
wiley +1 more source
Mitochondrial cardiolipin metabolism controlled by tafazzin enables ferroptosis
AbstractMitochondria are important producers of reactive oxygen species, which are involved in triggering ferroptosis, a lipid peroxidation driven form of cell death. Paradoxically, in the rare inherited metabolic disease Barth Syndrome, we discovered a protection from erastin-induced ferroptosis, despite intrinsically elevated mitochondrial ROS levels.
Wohlfarter Y +17 more
europepmc +2 more sources
Membrane Modulation and Cardiolipin Interaction of Small Molecules and Proteins [PDF]
165 Seiten, Illustrationen ...
Kleinwächter, Isabel
core +1 more source
Holding Out for a Model: Rhomboid Superfamily in Vertebrate Development and Disease
ABSTRACT The rhomboid superfamily, comprising both proteases and pseudoproteases, has emerged as a central regulator of membrane biology, mediating diverse functions including protein quality control, signal transduction, trafficking, and more. While molecular mechanisms of rhomboid activity have been well‐characterized in invertebrate and cell‐based ...
Saroj Gourkanti +7 more
wiley +1 more source
Barth Syndrome (BTHS) is a rare X-linked genetic disease caused by a mutation in the TAFAZZIN gene, which codes for the protein tafazzin involved in cardiolipin remodeling.
Hana M. Zegallai +2 more
doaj +1 more source
Ferroptosis in Pneumoconiosis: From Molecular and Cellular Mechanisms to Therapeutic Strategies
Targeting lung various cells to alleviate pneumoconiosis by suppressing ferroptosis. AAV9‐HGF, adeno‐associated virus‐hepatocyte growth factor; DFP, deferiprone; DHQ, dihydroquercetin; Fer‐1, ferrostatin‐1; IL‐1β, interleukin‐1β; NMN, nicotinamide mononucleotide; sh‐SLC39A14, shRNA targeting solute carrier family 39 member 14; si‐OC‐STAMP, siRNA ...
Mengli Yan +4 more
wiley +1 more source
Monolysocardiolipin in cultured fibroblasts is a sensitive and specific marker for Barth Syndrome
Barth Syndrome (BTHS) is an X-linked recessive disorder that results in abnormal metabolism of the mitochondrial phospholipid cardiolipin (CL). CLs are decreased and monolysocardiolipins (MLCLs), intermediates in CL metabolism, are increased in a variety
Michiel Adriaan van Werkhoven +3 more
doaj +1 more source
Mitochondria as Regulators of Nonapoptotic Cell Death in Cancer
Mitochondrial fission and fusion are key processes in maintaining cellular health. Fission is driven by proteins like, Fis‐1, which recruits DRP‐1, to facilitate the division of mitochondria. Fusion, however, is mediated by mitofusion 1(MFN1), mitofusion 2(MFN2), and optic atrophy 1 (OPA1), which work together to merge mitochondria, allowing functional
Saloni Malla +8 more
wiley +1 more source
Deficiency in Cardiolipin Reduces Doxorubicin-Induced Oxidative Stress and Mitochondrial Damage in Human B-Lymphocytes. [PDF]
Cardiolipin (CL) is an inner mitochondrial membrane phospholipid which plays an important role in mitochondrial function. Perturbation in CL biosynthesis alters mitochondrial bioenergetics causing a severe genetic disorder commonly known as Barth ...
Baikuntha Aryal, V Ashutosh Rao
doaj +1 more source
Molecular aspects of WNT/Frizzled signalling in brain angiogenesis [PDF]
The family of WNT lipoglycoproteins consists of 19 secreted proteins that are important for multiple cellular processes including cell proliferation, migration and fate.
Hot, Belma
core +1 more source

