Organization and expression of the mammalian mitochondrial genome

O Rackham, A Filipovska - Nature Reviews Genetics, 2022 - nature.com
The mitochondrial genome encodes core subunits of the respiratory chain that drives
oxidative phosphorylation and is, therefore, essential for energy conversion. Advances in …

Mechanisms and regulation of protein synthesis in mitochondria

E Kummer, N Ban - Nature Reviews Molecular Cell Biology, 2021 - nature.com
Mitochondria are cellular organelles responsible for generation of chemical energy in the
process called oxidative phosphorylation. They originate from a bacterial ancestor and …

Mitochondrial transcription and translation: overview

AR D'Souza, M Minczuk - Essays in biochemistry, 2018 - portlandpress.com
Mitochondria are the major source of ATP in the cell. Five multi-subunit complexes in the
inner membrane of the organelle are involved in the oxidative phosphorylation required for …

[HTML][HTML] Regulation of mammalian mitochondrial gene expression: recent advances

SF Pearce, P Rebelo-Guiomar, AR D'Souza… - Trends in biochemical …, 2017 - cell.com
Perturbation of mitochondrial DNA (mtDNA) gene expression can lead to human
pathologies. Therefore, a greater appreciation of the basic mechanisms of mitochondrial …

Oxidative stress and neuroinflammation potentiate each other to promote progression of dopamine neurodegeneration

J He, G Zhu, G Wang, F Zhang - Oxidative Medicine and …, 2020 - Wiley Online Library
Parkinson's disease (PD) is a chronic and complex disease of the central nervous system
(CNS). Progressive loss of dopamine (DA) neurons in midbrain substantia nigra is …

RNA decay machines: Deadenylation by the Ccr4–Not and Pan2–Pan3 complexes

E Wahle, GS Winkler - Biochimica et Biophysica Acta (BBA)-Gene …, 2013 - Elsevier
Shortening and removal of the 3′ poly (A) tail of mature mRNA by poly (A)-specific 3′
exonucleases (deadenylases) is the initial and often rate-limiting step in mRNA degradation …

Making proteins in the powerhouse

BM Hällberg, NG Larsson - Cell metabolism, 2014 - cell.com
Understanding regulation of mitochondrial DNA (mtDNA) expression is of considerable
interest given that mitochondrial dysfunction is important in human pathology and aging …

Loss-of-function mutations in MGME1 impair mtDNA replication and cause multisystemic mitochondrial disease

C Kornblum, TJ Nicholls, TB Haack, S Schöler… - Nature …, 2013 - nature.com
Known disease mechanisms in mitochondrial DNA (mtDNA) maintenance disorders alter
either the mitochondrial replication machinery (POLG, POLG2 and C10orf2),, or the …

Human mitochondrial RNA decay mediated by PNPase–hSuv3 complex takes place in distinct foci

LS Borowski, A Dziembowski… - Nucleic acids …, 2013 - academic.oup.com
RNA decay is usually mediated by protein complexes and can occur in specific foci such as
P-bodies in the cytoplasm of eukaryotes. In human mitochondria nothing is known about the …

LRPPRC/SLIRP suppresses PNPase-mediated mRNA decay and promotes polyadenylation in human mitochondria

T Chujo, T Ohira, Y Sakaguchi, N Goshima… - Nucleic acids …, 2012 - academic.oup.com
In human mitochondria, 10 mRNAs species are generated from a long polycistronic
precursor that is transcribed from the heavy chain of mitochondrial DNA, in theory yielding …