Table A.

Mitochondrial DNA-Associated Leigh Syndrome and NARP: Genes and Databases

GeneChromosome LocusProteinClinVar
MT-ATP6 Mitochondrion ATP synthase subunit a MT-ATP6
MT-CO3 Mitochondrion Cytochrome c oxidase subunit 3 MT-CO3
MT-ND1 Mitochondrion NADH-ubiquinone oxidoreductase chain 1 MT-ND1
MT-ND2 Mitochondrion NADH-ubiquinone oxidoreductase chain 2 MT-ND2
MT-ND3 Mitochondrion NADH-ubiquinone oxidoreductase chain 3 MT-ND3
MT-ND4 Mitochondrion NADH-ubiquinone oxidoreductase chain 4 MT-ND4
MT-ND5 Mitochondrion NADH-ubiquinone oxidoreductase chain 5 MT-ND5
MT-ND6 Mitochondrion NADH-ubiquinone oxidoreductase chain 6 MT-ND6
MT-TI MitochondrionNot applicable MT-TI
MT-TK MitochondrionNot applicable MT-TK
MT-TL1 MitochondrionNot applicable MT-TL1
MT-TL2 MitochondrionNot applicable MT-TL2
MT-TV MitochondrionNot applicable MT-TV
MT-TW MitochondrionNot applicable MT-TW

Data are compiled from the following standard references: gene from HGNC; chromosome locus from OMIM; protein from UniProt. For a description of databases (Locus Specific, HGMD, ClinVar) to which links are provided, click here.

From: Mitochondrial DNA-Associated Leigh Syndrome and NARP

Cover of GeneReviews®
GeneReviews® [Internet].
Adam MP, Mirzaa GM, Pagon RA, et al., editors.
Seattle (WA): University of Washington, Seattle; 1993-2023.
Copyright © 1993-2023, University of Washington, Seattle. GeneReviews is a registered trademark of the University of Washington, Seattle. All rights reserved.

GeneReviews® chapters are owned by the University of Washington. Permission is hereby granted to reproduce, distribute, and translate copies of content materials for noncommercial research purposes only, provided that (i) credit for source ( and copyright (© 1993-2023 University of Washington) are included with each copy; (ii) a link to the original material is provided whenever the material is published elsewhere on the Web; and (iii) reproducers, distributors, and/or translators comply with the GeneReviews® Copyright Notice and Usage Disclaimer. No further modifications are allowed. For clarity, excerpts of GeneReviews chapters for use in lab reports and clinic notes are a permitted use.

For more information, see the GeneReviews® Copyright Notice and Usage Disclaimer.

For questions regarding permissions or whether a specified use is allowed, contact: ude.wu@tssamda.

NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.