Detection of mitochondrial DNA (mtDNA) mutations.


Journal

Methods in cell biology
ISSN: 0091-679X
Titre abrégé: Methods Cell Biol
Pays: United States
ID NLM: 0373334

Informations de publication

Date de publication:
2020
Historique:
entrez: 19 3 2020
pubmed: 19 3 2020
medline: 29 12 2020
Statut: ppublish

Résumé

The maternally inherited mitochondrial DNA (mtDNA) is a circular 16,569bp double stranded DNA that encodes 37 genes, 24 of which (2 rRNAs and 22 tRNAs) are necessary for transcription and translation of 13 polypeptides that are all subunits of respiratory chain. Pathogenic mutations in mtDNA cause respiratory chain dysfunction, and are the underlying defect in an ever-increasing number of mtDNA-related encephalomyopathies with distinct phenotypes. In this chapter, we present an overview of mtDNA mutations and describe the molecular techniques currently employed in our laboratory to detect two types of mtDNA mutations: single-large-scale rearrangements and point mutations.

Identifiants

pubmed: 32183969
pii: S0091-679X(19)30139-6
doi: 10.1016/bs.mcb.2019.11.009
pmc: PMC7772943
mid: NIHMS1622166
pii:
doi:

Substances chimiques

DNA, Mitochondrial 0

Types de publication

Journal Article Research Support, N.I.H., Extramural

Langues

eng

Sous-ensembles de citation

IM

Pagination

383-400

Subventions

Organisme : NIGMS NIH HHS
ID : SC3 GM116669
Pays : United States

Informations de copyright

© 2020 Elsevier Inc. All rights reserved.

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Auteurs

Ali Naini (A)

Laboratory of Personalized Genomic Medicine, Department of Pathology and Cell Biology, Columbia University, New York, NY, United States; Houston Merritt Clinical Research Center for Inherited Myopathies and Mitochondrial Diseases, Department of Neurology, Columbia University Medical Center, New York, NY, United States. Electronic address: abn2@columbia.edu.

Robert Gilkerson (R)

Departments of Biology and Clinical Laboratory Sciences, The University of Texas Rio Grande Valley, Edinburg, TX, United States.

Sara Shanske (S)

Houston Merritt Clinical Research Center for Inherited Myopathies and Mitochondrial Diseases, Department of Neurology, Columbia University Medical Center, New York, NY, United States.

Jiuhong Pang (J)

Laboratory of Personalized Genomic Medicine, Department of Pathology and Cell Biology, Columbia University, New York, NY, United States.

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Classifications MeSH