A structure and function relationship study to identify the impact of the R721G mutation in the human mitochondrial lon protease.
Sign Up to like & getrecommendations! Published in 2021 at "Archives of biochemistry and biophysics"
DOI: 10.1016/j.abb.2021.108983
Abstract: Lon is an ATP-dependent protease belonging to the "ATPase associated with diverse cellular activities" (AAA+) protein family. In humans, Lon is translated as a precursor and imported into the mitochondria matrix through deletion of the… read more here.
Keywords: lon; protease; human mitochondrial; mitochondrial lon ... See more keywords
Clinical Therapeutic Management of Human Mitochondrial Disorders.
Sign Up to like & getrecommendations! Published in 2020 at "Pediatric neurology"
DOI: 10.1016/j.pediatrneurol.2020.07.004
Abstract: Despite recent advances in the elucidation of etiology and pathogenesis of mitochondrial disorders, their therapeutic management remains challenging. This review focuses on currently available therapeutic options for human mitochondrial disorders. Current treatment of mitochondrial disorders… read more here.
Keywords: human mitochondrial; mitochondrial disorders; deficiency; therapeutic management ... See more keywords
Human Mitochondrial Protein HSPD1 Binds to and Regulates the Repair of Deoxyinosine in DNA.
Sign Up to like & getrecommendations! Published in 2023 at "Journal of proteome research"
DOI: 10.1021/acs.jproteome.2c00854
Abstract: The generation of deoxyinosine (dI) in DNA is one of the most important sources of genetic mutations, which may lead to cancer and other human diseases. A further understanding of the biological consequences of dI… read more here.
Keywords: deoxyinosine dna; dna; hspd1; protein hspd1 ... See more keywords
Complete chemical structures of human mitochondrial tRNAs
Sign Up to like & getrecommendations! Published in 2020 at "Nature Communications"
DOI: 10.1038/s41467-020-18068-6
Abstract: Mitochondria generate most cellular energy via oxidative phosphorylation. Twenty-two species of mitochondrial (mt-)tRNAs encoded in mtDNA translate essential subunits of the respiratory chain complexes. mt-tRNAs contain post-transcriptional modifications introduced by nuclear-encoded tRNA-modifying enzymes. They are… read more here.
Keywords: mitochondrial trnas; human mitochondrial; trna modifications; chemical structures ... See more keywords
A late-stage assembly checkpoint of the human mitochondrial ribosome large subunit
Sign Up to like & getrecommendations! Published in 2022 at "Nature Communications"
DOI: 10.1038/s41467-022-28503-5
Abstract: Many cellular processes, including ribosome biogenesis, are regulated through post-transcriptional RNA modifications. Here, a genome-wide analysis of the human mitochondrial transcriptome shows that 2’- O -methylation is limited to residues of the mitoribosomal large subunit… read more here.
Keywords: stage assembly; large subunit; human mitochondrial; biogenesis ... See more keywords
Bedaquiline inhibits the yeast and human mitochondrial ATP synthases
Sign Up to like & getrecommendations! Published in 2020 at "Communications Biology"
DOI: 10.1038/s42003-020-01173-z
Abstract: Bedaquiline (BDQ, Sirturo) has been approved to treat multidrug resistant forms of Mycobacterium tuberculosis. Prior studies suggested that BDQ was a selective inhibitor of the ATP synthase from M. tuberculosis. However, Sirturo treatment leads to… read more here.
Keywords: human mitochondrial; mitochondrial atp; bdq; atp synthases ... See more keywords
Synthesis and properties of the anticodon stem-loop of human mitochondrial tRNAMet containing the disease-related G or m1G nucleosides at position 37.
Sign Up to like & getrecommendations! Published in 2021 at "Chemical communications"
DOI: 10.1039/d1cc05215b
Abstract: A single point mutation (A4435G) in the human mitochondrial tRNAMet (hmt-tRNAMet) gene causes severe mitochondrial disorders associated with hypertension, type 2 diabetes and LHON. This mutation leads to the exchange of A37 in the anticodon… read more here.
Keywords: mitochondrial trnamet; anticodon stem; human mitochondrial; stem loop ... See more keywords
A rapid method to visualize human mitochondrial DNA replication through rotary shadowing and transmission electron microscopy.
Sign Up to like & getrecommendations! Published in 2021 at "Nucleic acids research"
DOI: 10.1093/nar/gkab770
Abstract: We report a rapid experimental procedure based on high-density in vivo psoralen inter-strand DNA cross-linking coupled to spreading of naked purified DNA, positive staining, low-angle rotary shadowing, and transmission electron microscopy (TEM) that allows quick… read more here.
Keywords: dna; microscopy; human mitochondrial; mitochondrial dna ... See more keywords
Human mitochondrial transcription factors TFAM and TFB2M work synergistically in promoter melting during transcription initiation
Sign Up to like & getrecommendations! Published in 2017 at "Nucleic Acids Research"
DOI: 10.1093/nar/gkw1157
Abstract: Human mitochondrial DNA is transcribed by POLRMT with the help of two initiation factors, TFAM and TFB2M. The current model postulates that the role of TFAM is to recruit POLRMT and TFB2M to melt the… read more here.
Keywords: transcription; initiation; human mitochondrial; factors tfam ... See more keywords
AmtDB: a database of ancient human mitochondrial genomes
Sign Up to like & getrecommendations! Published in 2019 at "Nucleic Acids Research"
DOI: 10.1093/nar/gky843
Abstract: Abstract Ancient mitochondrial DNA is used for tracing human past demographic events due to its population-level variability. The number of published ancient mitochondrial genomes has increased in recent years, alongside with the development of high-throughput… read more here.
Keywords: amtdb database; ancient human; human mitochondrial; mitochondrial genomes ... See more keywords
A coordinated multiorgan metabolic response contributes to human mitochondrial myopathy.
Sign Up to like & getrecommendations! Published in 2023 at "EMBO molecular medicine"
DOI: 10.15252/emmm.202216951
Abstract: Mitochondrial diseases are a heterogeneous group of monogenic disorders that result from impaired oxidative phosphorylation (OXPHOS). As neuromuscular tissues are highly energy-dependent, mitochondrial diseases often affect skeletal muscle. Although genetic and bioenergetic causes of OXPHOS… read more here.
Keywords: human mitochondrial; metabolic response; coordinated multiorgan; mitochondrial myopathy ... See more keywords