LAUSR

About LAUSR
Donate
Contact Us

Follow us:

You are not signed in:

Sign Up!


   Articles with "human mitochondrial" as a keyword



Photo from wikipedia

A structure and function relationship study to identify the impact of the R721G mutation in the human mitochondrial lon protease.

Sign Up to like & get
recommendations! 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
Photo from wikipedia

Clinical Therapeutic Management of Human Mitochondrial Disorders.

Sign Up to like & get
recommendations! 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 & get
recommendations! 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
Photo by nci from unsplash

Complete chemical structures of human mitochondrial tRNAs

Sign Up to like & get
recommendations! 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 & get
recommendations! 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

Structural basis for substrate binding and selection by human mitochondrial RNA polymerase

Sign Up to like & get
recommendations! Published in 2024 at "Nature Communications"

DOI: 10.1038/s41467-024-50817-9

Abstract: The mechanism by which RNAP selects cognate substrates and discriminates between deoxy and ribonucleotides is of fundamental importance to the fidelity of transcription. Here, we present cryo-EM structures of human mitochondrial transcription elongation complexes that… read more here.

Keywords: site; structural basis; human mitochondrial; basis substrate ... See more keywords

Bedaquiline inhibits the yeast and human mitochondrial ATP synthases

Sign Up to like & get
recommendations! 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 & get
recommendations! 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

Mechanisms and pathologies of human mitochondrial DNA replication and deletion formation

Sign Up to like & get
recommendations! Published in 2024 at "Biochemical Journal"

DOI: 10.1042/bcj20230262

Abstract: Human mitochondria possess a multi-copy circular genome, mitochondrial DNA (mtDNA), that is essential for cellular energy metabolism. The number of copies of mtDNA per cell, and their integrity, are maintained by nuclear-encoded mtDNA replication and… read more here.

Keywords: replication; mtdna; deletion formation; human mitochondrial ... See more keywords

A rapid method to visualize human mitochondrial DNA replication through rotary shadowing and transmission electron microscopy.

Sign Up to like & get
recommendations! 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

The low endoribonuclease activity and lack of rNMP preference of human mitochondrial topoisomerase 1 protect against ribonucleotide-dependent deletions

Sign Up to like & get
recommendations! Published in 2025 at "Nucleic Acids Research"

DOI: 10.1093/nar/gkaf475

Abstract: Abstract The incorporation of ribonucleotides (rNMPs) into the nuclear genome leads to severe genomic instability, including strand breaks and short 2–5 bp deletions at repetitive sequences. Curiously, the detrimental effects of rNMPs are not observed… read more here.

Keywords: endoribonuclease activity; dependent deletions; rnmp; human mitochondrial ... See more keywords