Mitochondrial HMG-Box Containing Proteins: From Biochemical Properties to the Roles in Human Diseases

Autor: Veronika Vozáriková, Nina Kunová, Jacob A. Bauer, Ján Frankovský, Veronika Kotrasová, Katarína Procházková, Vladimíra Džugasová, Eva Kutejová, Vladimír Pevala, Jozef Nosek, Ľubomír Tomáška
Jazyk: angličtina
Rok vydání: 2020
Předmět:
Zdroj: Biomolecules, Vol 10, Iss 8, p 1193 (2020)
Druh dokumentu: article
ISSN: 2218-273X
DOI: 10.3390/biom10081193
Popis: Mitochondrial DNA (mtDNA) molecules are packaged into compact nucleo-protein structures called mitochondrial nucleoids (mt-nucleoids). Their compaction is mediated in part by high-mobility group (HMG)-box containing proteins (mtHMG proteins), whose additional roles include the protection of mtDNA against damage, the regulation of gene expression and the segregation of mtDNA into daughter organelles. The molecular mechanisms underlying these functions have been identified through extensive biochemical, genetic, and structural studies, particularly on yeast (Abf2) and mammalian mitochondrial transcription factor A (TFAM) mtHMG proteins. The aim of this paper is to provide a comprehensive overview of the biochemical properties of mtHMG proteins, the structural basis of their interaction with DNA, their roles in various mtDNA transactions, and the evolutionary trajectories leading to their rapid diversification. We also describe how defects in the maintenance of mtDNA in cells with dysfunctional mtHMG proteins lead to different pathologies at the cellular and organismal level.
Databáze: Directory of Open Access Journals
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