Results 81 to 90 of about 2,752 (180)
Role of Tafazzin and its isoforms on cardiolipin composition, cellular proliferation and gene expression [PDF]
Tafazzin is an acyltransferase with key functions in remodeling of the mitochondrial phospholipid cardiolipin (CL) by exchanging single fatty acids species in CL.
Jagirdar, Gayatri
core +1 more source
Role of mitophagy and mitochondrial quality-control in Barth syndrome.The Saccharomyces cerevisiae TAZ1 gene is an orthologue of human TAZ; both encode the protein tafazzin.
Gaspard, Gerard
core +2 more sources
Mitochondrial mislocalization and altered assembly of a cluster of Barth syndrome mutant tafazzins [PDF]
None of the 28 identified point mutations in tafazzin (Taz1p), which is the mutant gene product associated with Barth syndrome (BTHS), has a biochemical explanation. In this study, endogenous Taz1p was localized to mitochondria in association with both the inner and outer mitochondrial membranes facing the intermembrane space (IMS). Unexpectedly, Taz1p
Claypool, Steven M +2 more
openaire +4 more sources
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
Immunometabolic and Spatiotemporal Control of Tissue‐Resident Memory T Cell Biology
Tissue‐resident memory T (TRM) cells in barrier tissues provide a frontline defense against invading pathogens. Immune (Signals 1–3) and nutrient (Signal 4) cues play an integral role in directing TRM formation and heterogeneity. The spatial and temporal organization of these signals establishes durable TRM cells across tissues, enabling diverse ...
Jana L. Raynor, Hongbo Chi
wiley +1 more source
Mitochondrial Transplantation as a Therapeutic Strategy for Inherited Mitochondrial Diseases
Mitochondrial transplantation (MTx) offers a promising therapeutic avenue for mitochondrial diseases. This review comprehensively evaluates MTx, differentiating its feasibility for mtDNA‐ and nDNA‐based disorders. It examines its potential for genetic correction, alongside inherent limitations, technical challenges, and crucial ethical considerations ...
Parmeshar Singh +17 more
wiley +1 more source
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
Mutations in the gene encoding the enzyme tafazzin, TAZ, cause Barth syndrome (BTHS). Individuals with this X-linked multisystem disorder present cardiomyopathy (often dilated), skeletal muscle weakness, neutropenia, growth retardation and 3 ...
Ana eSaric +5 more
doaj +1 more source
Introduction The case report describes a novel finding of a homozygous variant in the coronin 1A (CORO1A) gene, associated with atypical severe combined immunodeficiency (SCID) in a 9‐year‐old female patient with recurrent infections and unique immunological features, including periodic T‐cell lymphocytosis and T‐ and B‐cell lymphopenia.
Alanoud Aljohani +6 more
wiley +1 more source
Tafazzin knockdown and Dox-sensitivity in H9c2 cardiomyocytes.
(A) knockdown of tafazzin was confirmed in H9c2 cells after transfection with 20 nM tafazzin siRNA for 48 h by western blot analysis. (B) Representative western blot showing the level of cleaved caspase-3.
Baikuntha Aryal (441940) +1 more
core +1 more source

