Search Results - "GOHIL, Vishal M"
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The role of nonbilayer phospholipids in mitochondrial structure and function
Published in FEBS letters (01-04-2018)“…Mitochondrial structure and function are influenced by the unique phospholipid composition of its membranes. While mitochondria contain all the major classes…”
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FDX1-dependent and independent mechanisms of elesclomol-mediated intracellular copper delivery
Published in Proceedings of the National Academy of Sciences - PNAS (07-03-2023)“…Recent studies have uncovered the therapeutic potential of elesclomol (ES), a copper-ionophore, for copper deficiency disorders. However, we currently do not…”
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Elesclomol alleviates Menkes pathology and mortality by escorting Cu to cuproenzymes in mice
Published in Science (American Association for the Advancement of Science) (08-05-2020)“…Loss-of-function mutations in the copper (Cu) transporter ATP7A cause Menkes disease. Menkes is an infantile, fatal, hereditary copper-deficiency disorder that…”
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Specific requirements of nonbilayer phospholipids in mitochondrial respiratory chain function and formation
Published in Molecular biology of the cell (15-07-2016)“…Mitochondrial membrane phospholipid composition affects mitochondrial function by influencing the assembly of the mitochondrial respiratory chain (MRC)…”
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The Role of COA6 in the Mitochondrial Copper Delivery Pathway to Cytochrome c Oxidase
Published in Biomolecules (Basel, Switzerland) (13-01-2022)“…Copper is essential for the stability and activity of cytochrome oxidase (CcO), the terminal enzyme of the mitochondrial respiratory chain. Copper is bound to…”
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Mitochondrial dysfunctions in barth syndrome
Published in IUBMB life (01-07-2019)“…ABSTRACT Barth syndrome (BTHS) is a rare multisystemic genetic disorder caused by mutations in the TAZ gene. TAZ encodes a mitochondrial enzyme that remodels…”
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Atomic vacancies of molybdenum disulfide nanoparticles stimulate mitochondrial biogenesis
Published in Nature communications (17-09-2024)“…Diminished mitochondrial function underlies many rare inborn errors of energy metabolism and contributes to more common age-associated metabolic and…”
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A computational screen for regulators of oxidative phosphorylation implicates SLIRP in mitochondrial RNA homeostasis
Published in PLoS genetics (01-08-2009)“…The human oxidative phosphorylation (OxPhos) system consists of approximately 90 proteins encoded by nuclear and mitochondrial genomes and serves as the…”
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Vps39 is required for ethanolamine-stimulated elevation in mitochondrial phosphatidylethanolamine
Published in Biochimica et biophysica acta. Molecular and cell biology of lipids (01-06-2020)“…Mitochondrial membrane biogenesis requires the import of phospholipids; however, the molecular mechanisms underlying this process remain elusive. Recent work…”
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COA6 Is Structurally Tuned to Function as a Thiol-Disulfide Oxidoreductase in Copper Delivery to Mitochondrial Cytochrome c Oxidase
Published in Cell reports (Cambridge) (17-12-2019)“…In eukaryotes, cellular respiration is driven by mitochondrial cytochrome c oxidase (CcO), an enzyme complex that requires copper cofactors for its catalytic…”
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Yeast homologs of human MCUR1 regulate mitochondrial proline metabolism
Published in Nature communications (25-09-2020)“…Mitochondria house evolutionarily conserved pathways of carbon and nitrogen metabolism that drive cellular energy production. Mitochondrial bioenergetics is…”
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Mitochondrial dysfunction reactivates α-fetoprotein expression that drives copper-dependent immunosuppression in mitochondrial disease models
Published in The Journal of clinical investigation (03-01-2023)“…Signaling circuits crucial to systemic physiology are widespread, yet uncovering their molecular underpinnings remains a barrier to understanding the etiology…”
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13
Ethanolamine ameliorates mitochondrial dysfunction in cardiolipin-deficient yeast cells
Published in The Journal of biological chemistry (13-07-2018)“…Cardiolipin (CL) is a signature phospholipid of the mitochondria required for the formation of mitochondrial respiratory chain (MRC) supercomplexes. The…”
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Editorial: Function and formation of mitochondrial metalloproteome
Published in Frontiers in cell and developmental biology (28-09-2022)Get full text
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15
Noncanonical role of singleminded-2s in mitochondrial respiratory chain formation in breast cancer
Published in Experimental & molecular medicine (01-05-2023)“…Dysregulation of cellular metabolism is a hallmark of breast cancer progression and is associated with metastasis and therapeutic resistance. Here, we show…”
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Repurposing elesclomol, an investigational drug for the treatment of copper metabolism disorders
Published in Expert opinion on investigational drugs (02-01-2021)Get more information
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Synthetic Lethal Interaction of the Mitochondrial Phosphatidylethanolamine and Cardiolipin Biosynthetic Pathways in Saccharomyces cerevisiae
Published in The Journal of biological chemistry (21-10-2005)“…Saccharomyces cerevisiae mitochondria contain enzymes required for synthesis of the phospholipids cardiolipin (CL) and phosphatidylethanolamine (PE), which are…”
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Cardiolipin deficiency leads to the destabilization of mitochondrial magnesium channel MRS2 in Barth syndrome
Published in Human molecular genetics (01-12-2023)“…Abstract Barth syndrome (BTHS) is a debilitating X-linked cardio-skeletal myopathy caused by loss-of-function mutations in TAFAZZIN, a cardiolipin…”
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Molecular nature and physiological role of the mitochondrial calcium uniporter channel
Published in American Journal of Physiology: Cell Physiology (01-04-2021)“…Calcium (Ca ) signaling is critical for cell function and cell survival. Mitochondria play a major role in regulating the intracellular Ca concentration ([Ca ]…”
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Cardiolipin Biosynthesis and Mitochondrial Respiratory Chain Function Are Interdependent
Published in The Journal of biological chemistry (08-10-2004)“…Cardiolipin (CL) is an acidic phospholipid present almost exclusively in membranes harboring respiratory chain complexes. We have previously shown that, in…”
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