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Article

Shy1p is necessary for full expression of mitochondrial COX1 in the yeast model of Leigh's syndrome.

The EMBO journal - 15 Jan 2002

Barrientos Antoni, Korr Daniel, Tzagoloff Alexander

Abstract excerpt

SHY1 codes for a mitochondrial protein required for full expression of cytochrome oxidase (COX) in Saccharomyces cerevisiae. Mutations in the homologous human gene (SURF1) have been reported to cause Leigh's syndrome, a neurological disease associated with COX deficiency. The function of Shy1p/Surf1p is poorly understood. Here we have characterized revertants of shy1 null mutants carrying extragenic nuclear...

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Source

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Identifiers

PMID: 11782424
DOI: 10.1093/emboj/21.1.43
PMCID: PMC125806

Genes

MSS51
SURF1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

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Shy1p is necessary for full expression of mitochondrial COX1 in the yeast model of Leigh's syndrome.PMID 11782424Read ↗

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Transcriptional activators HAP/NF-Y rescue a cytochrome c oxidase defect in yeast and human cells.Semantic proximity Read ↗

Mutations of SURF-1 in Leigh disease associated with cytochrome c oxidase deficiency.Semantic proximity Read ↗

Nuclear gene defects in mitochondrial disorders.Semantic proximity Read ↗

Adaptation of respiratory chain biogenesis to cytochrome c oxidase deficiency caused by SURF1 gene mutations.Semantic proximity Read ↗

SHY1, the yeast homolog of the mammalian SURF-1 gene, encodes a mitochondrial protein required for respiration.Semantic proximity Read ↗

Leigh syndrome in Drosophila melanogaster: morphological and biochemical characterization of Surf1 post-transcriptional silencing.Semantic proximity Read ↗

The yeast nuclear gene MRF1 encodes a mitochondrial peptide chain release factor and cures several mitochondrial RNA splicing defects.Semantic proximity Read ↗

Mss51p, a putative translational activator of cytochrome c oxidase subunit-1 (COX1) mRNA, is required for synthesis of Cox1p in Saccharomyces cerevisiae.Semantic proximity Read ↗

Constitutive knockout of Surf1 is associated with high embryonic lethality, mitochondrial disease and cytochrome c oxidase deficiency in mice.Semantic proximity Read ↗

SURF1, encoding a factor involved in the biogenesis of cytochrome c oxidase, is mutated in Leigh syndrome.Semantic proximity Read ↗

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