Erin L. Seifert

Rescue of the First Mitochondrial Membrane Carrier, the mPiC, by TAT-Mediated Protein Replacement Treatment

Samar Zabit, Orly Melloul, Michal Lichtenstein, Erin L. Seifert et autres

The mitochondrial phosphate carrier (mPiC), encoded by the nuclear gene SLC25A3, is synthesized with an N-terminus mitochondrial targeting sequence (MTS), enabling its import into the mitochondria. mPiC imports inorganic phosphate (Pi) into the mitochondrial matrix for ATP production and other matrix phosphorylation …

il, us (code pays fourni par la source)

Individualized exercise and NAD+ precursor supplementation in Friedreich’s Ataxia: a randomized controlled trial

Kimberly Y. Lin, Anna Bucha, K. McSweeney, Kristin Wade et autres

us (code pays fourni par la source)

Post-transcriptional methylation of mitochondrial-tRNA differentially contributes to mitochondrial pathology

Sunita Maharjan, Howard Gamper, Yuka Yamaki, Thomas Christian et autres

Human mitochondrial tRNAs (mt-tRNAs), critical for mitochondrial biogenesis, are frequently associated with pathogenic mutations. These mt-tRNAs have unusual sequence motifs and require post-transcriptional modifications to stabilize their fragile structures. However, whether a modification that stabilizes a wild-type (WT) mt-tRNA would also stabilize …

us, jp (code pays fourni par la source)

Mitochondrial phosphate carrier depletion in skeletal muscle and Ca2+ homeostasis

Erin L. Seifert, György Hajnóczky, C. Vásquez, Valentina Debattisti et autres

Acyl-CoA thioesterase-2 facilitates β-oxidation in glycolytic skeletal muscle in a lipid supply dependent manner

Carmen Bekeová, Ji In Han, He-Li Xu, Evan W. Kerr et autres

Abstract Acyl-Coenzyme A (acyl-CoA) thioesters are compartmentalized intermediates that participate in in multiple metabolic reactions within the mitochondrial matrix. The limited availability of free CoA (CoASH) in the matrix raises the question of how the local acyl-CoA concentration is regulated to prevent …

us, it, de (code pays fourni par la source)

Acyl-CoA thioesterase-2 facilitates β-oxidation in glycolytic skeletal muscle in a lipid supply dependent manner

Carmen Bekeová, Ji In Han, He-Li Xu, Evan W. Kerr et autres

Abstract Acyl-Coenzyme A (acyl-CoA) thioesters are compartmentalized intermediates that participate in in multiple metabolic reactions within the mitochondrial matrix. The limited availability of free CoA (CoASH) in the matrix raises the question of how the local acyl-CoA concentration is regulated to prevent …

us, it, de (code pays fourni par la source)

Post-Transcriptional Methylation of Mitochondrial-tRNA Differentially Contributes to Mitochondrial Pathology

Sunita Maharjan, Howard Gamper, Yuka Yamaki, Robert Y. Henley et autres

SUMMARY Human mitochondrial tRNAs (mt-tRNAs), critical for mitochondrial biogenesis, are frequently associated with pathogenic mutations. These mt-tRNAs have unusual sequence motifs and require post-transcriptional modifications to stabilize their fragile structures. However, whether a modification that stabilizes a wild-type (WT) mt-tRNA structure would …

us, jp (code pays fourni par la source)

Comparison of colorimetric, fluorometric, and liquid chromatography-mass spectrometry assays for acetyl-coenzyme A

Daniel S. Kantner, Emily Megill, Anna Bostwick, Vicky K. Yang et autres

us (code pays fourni par la source)

Erratum To: MSTO1 is a cytoplasmic pro‐mitochondrial fusion protein, whose mutation induces myopathy and ataxia in humans

Anikó Gál, Péter Balicza, David Weaver, Shamim Naghdi et autres

Graphical Abstract

Acyl-CoA thioesterase-2 facilitates β-oxidation in glycolytic skeletal muscle in a lipid supply dependent manner

Carmen Bekeová, Ji In Han, He-Li Xu, Evan W. Kerr et autres

ABSTRACT Acyl-Coenzyme A (acyl-CoA) thioesters are compartmentalized intermediates that participate in in multiple metabolic reactions within the mitochondrial matrix. The limited availability of free CoA (CoASH) in the matrix raises the question of how the local acyl-CoA concentration is regulated to prevent …

us, it, de (code pays fourni par la source)

Comparison of colorimetric, fluorometric, and liquid chromatography-mass spectrometry assays for acetyl-coenzyme A

Daniel S. Kantner, Emily Megill, Anna Bostwick, Vicky K. Yang et autres

Acetyl-Coenzyme A is a central metabolite in catabolic and anabolic pathways as well as the acyl donor for acetylation reactions. Multiple quantitative measurement techniques for acetyl-CoA have been reported, including commercially available kits. Comparisons between techniques for acetyl-CoA measurement have not been …

us (code pays fourni par la source)

Fuel oxidation under the control of mitochondrial shape and dynamics

Erin L. Seifert, György Hajnóczky

How mitochondrial shape and substrate-specific metabolism are related has been a difficult question to address. Here, new work by Ngo et al (2023) reports that mitochondrial shape-long versus fragmented-determines the activity of β-oxidation of long-chain fatty acids, supporting a novel role for …

us (code pays fourni par la source)