Journal ArticleDOI
Functional delivery of a cytosolic tRNA into mutant mitochondria of human cells
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TLDR
It is found that the Leishmania RNA import complex (RIC) could enter human cells by a caveolin-1–dependent pathway, where it induced import of endogenous cytosolic tRNAs, including tRNALys, and restored mitochondrial function in a cybrid harboring a mutant mt tRNalys (MT-TK) gene.Abstract:
Many maternally inherited and incurable neuromyopathies are caused by mutations in mitochondrial (mt) transfer RNA (tRNA) genes. Kinetoplastid protozoa, including Leishmania, have evolved specialized systems for importing nucleus-encoded tRNAs into mitochondria. We found that the Leishmania RNA import complex (RIC) could enter human cells by a caveolin-1-dependent pathway, where it induced import of endogenous cytosolic tRNAs, including tRNA(Lys), and restored mitochondrial function in a cybrid harboring a mutant mt tRNA(Lys) (MT-TK) gene. The use of protein complexes to modulate mitochondrial function may help in the management of such genetic disorders.read more
Citations
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Inflammation and metabolic disorders
TL;DR: Dysfunction of the immune response and metabolic regulation interface can be viewed as a central homeostatic mechanism, dysfunction of which can lead to a cluster of chronic metabolic disorders, particularly obesity, type 2 diabetes and cardiovascular disease.
Journal ArticleDOI
Mitochondrial membrane potential.
Ljubava D. Zorova,Vasily A. Popkov,Egor Y. Plotnikov,Denis N. Silachev,Irina B. Pevzner,S. S. Jankauskas,V. A. Babenko,S. D. Zorov,Anastasia V. Balakireva,Magdalena Juhaszova,Steven J. Sollott,Dmitry B. Zorov,Dmitry B. Zorov +12 more
TL;DR: Additional potential mechanisms for which ΔΨm is essential for maintenance of cellular health and viability are proposed and recommendations how to accurately measure ΔΩm in a cell are provided and potential sources of artifacts are discussed.
Journal ArticleDOI
Mitochondrial Energetics and Therapeutics
TL;DR: Analysis of the many ways that a shift from carbohydrate glycolytic metabolism to fatty acid and ketone oxidative metabolism may modulate metabolism, signal transduction pathways, and the epigenome gives an appreciation of the ketogenic diet and the potential for bioenergetic therapeutics.
Journal ArticleDOI
Mitochondrial defects and oxidative stress in Alzheimer disease and Parkinson disease.
TL;DR: Changes in mitochondrial DNA and mitochondrial dynamics, two aspects critical to the maintenance of mitochondrial homeostasis and function, in relationship with oxidative stress in the pathogenesis of AD and PD are focused on.
Journal ArticleDOI
Genomic and biochemical insights into the specificity of ETS transcription factors.
TL;DR: The current knowledge of routes to functional diversity and DNA binding specificity is presented, including divergent properties of the conserved ETS and PNT domains, the involvement of flanking structured and unstructured regions appended to these dynamic domains, posttranslational modifications, and protein partnerships with other DNA-binding proteins and coregulators.
References
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Journal ArticleDOI
A mutation in the tRNA Leu(UUR) gene associated with the MELAS subgroup of mitochondrial encephalomyopathies
TL;DR: An A-to-G transition mutation at nucleotide pair 3,243 in the dihydrouridine loop of mitochondrial tRNALeu(UUR) that is specific to patients with MELAS is reported, which creates an Apal restriction site and could perform a simple molecular diagnostic test for the disease.
Journal ArticleDOI
Myoclonic epilepsy and ragged-red fiber disease (MERRF) is associated with a mitochondrial DNA tRNALys mutation
John M. Shoffner,Marie T. Lott,Angela M. S. Lezza,Peter Seibel,Scott W. Ballinger,Douglas C. Wallace +5 more
TL;DR: An A to G transition mutation at nucleotide pair 8344 in human mitochondrial DNA has been identified as the cause of MERRF, providing molecular confirmation that some forms of epilepsy are the result of deficiencies in mitochondrial energy production.
Journal ArticleDOI
Clathrin-independent endocytosis of ubiquitinated cargos
Sara Sigismund,Tanja Woelk,Claudia Puri,Elena Maspero,Carlo Tacchetti,Pietro Transidico,Pier Paolo Di Fiore,Simona Polo +7 more
TL;DR: It is shown that the epidermal growth factor (EGF) receptor (EGFR), when stimulated with low doses of EGF, is internalized almost exclusively through the clathrin pathway, and it is not ubiquitinated.
Journal ArticleDOI
Caveolae/raft-dependent endocytosis.
Ivan R. Nabi,Phuong U. Le +1 more
TL;DR: It is proposed that caveolae and rafts are internalized via a common pathway, Caveolae/raft-dependent endocytosis, defined by its clathrin independence, dynamin dependence, and sensitivity to cholesterol depletion.
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