Defects in mitochondrial protein synthesis and respiratory chain activity segregate with the tRNA(Leu(UUR)) mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes.

doi:10.1128/mcb.12.2.480-490.1992

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Article

Defects in mitochondrial protein synthesis and respiratory chain activity segregate with the tRNA(Leu(UUR)) mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes.

Molecular and cellular biology - 1 Feb 1992

King M P, Koga Y, Davidson M, Schon E A

Abstract excerpt

Cytoplasts from two unrelated patients with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes) harboring an A----G transition at nucleotide position 3243 in the tRNA(Leu(UUR)) gene of the mitochondrial genome were fused with human cells lacking endogenous mitochondrial DNA (mtDNA) (rho 0 cells). Selected cybrid lines, containing less than 15 or greater than or equal to 95%...

Topics

Acidosis, Lactic
Adolescent
Blotting, Northern
Brain Diseases, Metabolic
Cell Line
Cerebrovascular Disorders
Child
DNA, Mitochondrial
Female
Humans
Hybrid Cells

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Immunohistochemistry

Mitochondria

Mutation

Oxygen Consumption

Protein Biosynthesis

RNA, Transfer, Leu

Syndrome

Source

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Identifiers

PMID: 1732728
DOI: 10.1128/mcb.12.2.480-490.1992
PMCID: PMC364194

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 29 Aug 2026. Prepared record publication-1732728-2026-08-29.1. Manifest publication-graph-cohort-2026-08-29.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 29 Aug 2026.

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Defects in mitochondrial protein synthesis and respiratory chain activity segregate with the tRNA(Leu(UUR)) mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes.PMID 1732728Read ↗

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Pathophysiology of the MELAS 3243 transition mutation.Semantic proximity Read ↗

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