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Article

Human NARP mitochondrial mutation metabolism corrected with alpha-ketoglutarate/aspartate: a potential new therapy.

Archives of neurology - 1 Aug 2009

Sgarbi Gianluca, Casalena Gabriella A, Baracca Alessandra, Lenaz Giorgio, DiMauro Salvatore, Solaini Giancarlo

Abstract excerpt

OBJECTIVE: To verify whether enhanced substrate-level phosphorylation increases viability and adenosine 5'-triphosphate (ATP) content of cells with neuropathy, ataxia, and retinitis pigmentosa/maternally inherited Leigh syndrome (NARP/MILS) mitochondrial DNA mutations and ATP synthase dysfunction. DESIGN: We used cell lines "poisoned" with oligomycin, the specific inhibitor of ATP synthase, and "natural" models,...

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Identifiers

PMID: 19667215
DOI: 10.1001/archneurol.2009.134

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

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