Mitochondrial heat shock protein 70, a molecular chaperone for proteins encoded by mitochondrial DNA [PDF]
Mitochondrial heat shock protein 70 (mt-Hsp70) has been shown to play an important role in facilitating import into, as well as folding and assembly of nuclear-encoded proteins in the mitochondrial matrix.
W Neupert +7 more
core +1 more source
mtDNA heteroplasmy level and copy number indicate disease burden in m.3243A>G mitochondrial disease
Mitochondrial disease associated with the pathogenic m.3243A>G variant is a common, clinically heterogeneous, neurogenetic disorder. Using multiple linear regression and linear mixed modelling, we evaluated which commonly assayed tissue (blood N = 231 ...
John P Grady +13 more
doaj +1 more source
Mitochondrial helicases and mitochondrial genome maintenance [PDF]
Helicases are essential enzymes that utilize the energy of nucleotide hydrolysis to drive unwinding of nucleic acid duplexes. Helicases play roles in all aspects of DNA metabolism including DNA repair, DNA replication and transcription. The subcellular locations and functions of several helicases have been studied in detail; however, the roles of ...
de Souza-Pinto, Nadja C +4 more
openaire +3 more sources
Experimental Relocation of the Mitochondrial ATP9 Gene to the Nucleus Reveals Forces Underlying Mitochondrial Genome Evolution [PDF]
Only a few genes remain in the mitochondrial genome retained by every eukaryotic organism that carry out essential functions and are implicated in severe diseases.
Steinmetz⁎, L.M. +77 more
core +1 more source
Transport of proteins across mitochondrial membranes [PDF]
The vast majority of proteins comprising the mitochondrion are encoded by nuclear genes, synthesized on ribosomes in the cytosol, and translocated into the various mitochondrial subcompartments. During this process proteins must cross the lipid membranes
Neupert, Walter
core +1 more source
Mitochondrial dynamics: quantifying mitochondrial fusion in vitro [PDF]
Mitochondrial fusion is an essential process for preserving the integrity and stability of mitochondrial DNA; however, regulation of this process remains largely mysterious. In this issue of BMC Biology, Schauss and colleagues describe a simple, reliable, and robust novel assay that allows fusion of mammalian mitochondria to be quantified in vitro.
Jourdain Alexis, Martinou Jean-Claude
openaire +6 more sources
Altering the redox state of cysteine residues on protein surfaces is an important response to environmental challenges. Although aging and fasting alter many redox processes, the role of cysteine residues is uncertain.
Katja E. Menger +8 more
doaj +1 more source
Parkinson’s disease neurons exhibit alterations in mitochondrial quality control proteins
Mitochondrial dysfunction has been suggested to contribute to Parkinson’s disease pathogenesis, though an understanding of the extent or exact mechanism of this contribution remains elusive.
Chun Chen +14 more
doaj +1 more source
Codon optimization is an essential parameter for the efficient allotopic expression of mtDNA genes
Mutations in mitochondrial DNA can be inherited or occur de novo leading to several debilitating myopathies with no curative option and few or no effective treatments.
Caitlin J. Lewis +5 more
doaj +1 more source
Mitochondrial Protein Import [PDF]
The role of nucleoside triphosphates (NTPs) in mitochondrial protein import was investigated with the precursors of N. crassa ADP/ATP carrier, F1-ATPase subunit β, F0-ATPase subunit 9, and fusion proteins between subunit 9 and mouse dihydrofolate ...
Pfanner, Nikolaus +2 more
core +1 more source

