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Article

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

The Journal of biological chemistry - 17 Oct 2014

Da-Rè Caterina, von Stockum Sophia, Biscontin Alberto, Millino Caterina, Cisotto Paola, Zordan Mauro A, Zeviani Massimo, Bernardi Paolo, De Pittà Cristiano, Costa Rodolfo

Abstract excerpt

Leigh Syndrome (LS) is the most common early-onset, progressive mitochondrial encephalopathy usually leading to early death. The single most prevalent cause of LS is occurrence of mutations in the SURF1 gene, and LS(Surf1) patients show a ubiquitous and specific decrease in the activity of mitochondrial respiratory chain complex IV (cytochrome c oxidase, COX). SURF1 encodes an inner membrane mitochondrial protein...

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Source

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Identifiers

PMID: 25164807
DOI: 10.1074/jbc.M114.602938
PMCID: PMC4200275

Genes

SURF1

Provenance

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Source receipt: pubmed, retrieved 7 Sept 2026.

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Leigh syndrome in Drosophila melanogaster: morphological and biochemical characterization of Surf1 post-transcriptional silencing.PMID 25164807Read ↗

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