In vitro genetic transfer of protein synthesis and respiration defects to mitochondrial DNA-less cells with myopathy-patient mitochondria.
about
Molecular cloning, expression, and partial characterization of two novel members of the ovalbumin family of serine proteinase inhibitorsMitochondrial tRNA mutations and diseaseModification defect at anticodon wobble nucleotide of mitochondrial tRNAs(Leu)(UUR) with pathogenic mutations of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodesMitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS): current conceptsWobble modification defect in tRNA disturbs codon-anticodon interaction in a mitochondrial diseaseThe mtDNA-encoded ND6 subunit of mitochondrial NADH dehydrogenase is essential for the assembly of the membrane arm and the respiratory function of the enzyme.Physiological role of the N-terminal processed P4501A1 targeted to mitochondria in erythromycin metabolism and reversal of erythromycin-mediated inhibition of mitochondrial protein synthesisExpanded phenotypic spectrum of the m.8344A>G "MERRF" mutation: data from the German mitoNET registry.Impaired ATP synthase assembly associated with a mutation in the human ATP synthase subunit 6 gene.Mitochondrial DNA and primary mitochondrial dysfunction in Parkinson's disease.Reverse genetic studies of mitochondrial DNA-based diseases using a mouse model.Two novel pathogenic mitochondrial DNA mutations affecting organelle number and protein synthesis. Is the tRNA(Leu(UUR)) gene an etiologic hot spot?Clinical mitochondrial genetics.Random genetic drift determines the level of mutant mtDNA in human primary oocytesGene therapy for progeny of mito-mice carrying pathogenic mtDNA by nuclear transplantationMitochondrial NAD-dependent methylenetetrahydrofolate dehydrogenase-methenyltetrahydrofolate cyclohydrolase is essential for embryonic developmentAminoacylation properties of pathology-related human mitochondrial tRNA(Lys) variants.The myoclonic epilepsy and ragged-red fiber mutation provides new insights into human mitochondrial function and genetics.A stop-codon mutation in the human mtDNA cytochrome c oxidase I gene disrupts the functional structure of complex IV.Complete repopulation of mouse mitochondrial DNA-less cells with rat mitochondrial DNA restores mitochondrial translation but not mitochondrial respiratory function.Rearrangements of human mitochondrial DNA (mtDNA): new insights into the regulation of mtDNA copy number and gene expressionMitochondrial DNA: impacting central and peripheral nervous systems.Mitochondrial threshold effects.Mutations in mitochondrial tRNA genes: non-linkage with syndromes of Wolfram and chronic progressive external ophthalmoplegiaMultiple symmetric lipomas with high levels of mtDNA with the tRNA(Lys) A-->G(8344) mutation as the only manifestation of disease in a carrier of myoclonus epilepsy and ragged-red fibers (MERRF) syndrome.The mitochondrial tRNA(Leu(UUR)) mutation in mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS): genetic, biochemical, and morphological correlations in skeletal muscle.Segregation and manifestations of the mtDNA tRNA(Lys) A-->G(8344) mutation of myoclonus epilepsy and ragged-red fibers (MERRF) syndromeDistribution and threshold expression of the tRNA(Lys) mutation in skeletal muscle of patients with myoclonic epilepsy and ragged-red fibers (MERRF).Disorders of mitochondrial protein synthesis.A new mtDNA mutation showing accumulation with time and restriction to skeletal muscleDefects of mitochondrial DNA.Intracellular mitochondrial triplasmy in a patient with two heteroplasmic base changesMitochondrial DNA and genetic disease.The NADH:ubiquinone oxidoreductase (complex I) of respiratory chains.Congenital encephalomyopathy and adult-onset myopathy and diabetes mellitus: different phenotypic associations of a new heteroplasmic mtDNA tRNA glutamic acid mutation.Cytoplasmic transfer of the mtDNA nt 8993 T-->G (ATP6) point mutation associated with Leigh syndrome into mtDNA-less cells demonstrates cosegregation with a decrease in state III respiration and ADP/O ratio.The awakening of an advanced malignant cancer: an insult to the mitochondrial genomeMitochondrial DNA sequence variation in human evolution and disease.Structure and function of the mitochondrial genome.Platelet-mediated transformation of mtDNA-less human cells: analysis of phenotypic variability among clones from normal individuals--and complementation behavior of the tRNALys mutation causing myoclonic epilepsy and ragged red fibers.
P2860
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P2860
In vitro genetic transfer of protein synthesis and respiration defects to mitochondrial DNA-less cells with myopathy-patient mitochondria.
description
1991 nî lūn-bûn
@nan
1991年の論文
@ja
1991年論文
@yue
1991年論文
@zh-hant
1991年論文
@zh-hk
1991年論文
@zh-mo
1991年論文
@zh-tw
1991年论文
@wuu
1991年论文
@zh
1991年论文
@zh-cn
name
In vitro genetic transfer of p ...... myopathy-patient mitochondria.
@ast
In vitro genetic transfer of p ...... myopathy-patient mitochondria.
@en
type
label
In vitro genetic transfer of p ...... myopathy-patient mitochondria.
@ast
In vitro genetic transfer of p ...... myopathy-patient mitochondria.
@en
prefLabel
In vitro genetic transfer of p ...... myopathy-patient mitochondria.
@ast
In vitro genetic transfer of p ...... myopathy-patient mitochondria.
@en
P2093
P2860
P356
P1476
In vitro genetic transfer of p ...... myopathy-patient mitochondria.
@en
P2093
P2860
P304
P356
10.1128/MCB.11.4.2236
P407
P577
1991-04-01T00:00:00Z