Limited dCTP availability accounts for mitochondrial DNA depletion in mitochondrial neurogastrointestinal encephalomyopathy (MNGIE)
about
Nucleotide salvage deficiencies, DNA damage and neurodegenerationThe NTP pyrophosphatase DCTPP1 contributes to the homoeostasis and cleansing of the dNTP pool in human cellsLiver as a source for thymidine phosphorylase replacement in mitochondrial neurogastrointestinal encephalomyopathyLong term expression of Drosophila melanogaster nucleoside kinase in thymidine kinase 2-deficient mice with no lethal effects caused by nucleotide pool imbalances.MPV17 Loss Causes Deoxynucleotide Insufficiency and Slow DNA Replication in MitochondriaCyclin D3-dependent control of the dNTP pool and HIV-1 replication in human macrophagesNucleoside salvage pathway kinases regulate hematopoiesis by linking nucleotide metabolism with replication stress.Infantile Progressive Hepatoencephalomyopathy with Combined OXPHOS Deficiency due to Mutations in the Mitochondrial Translation Elongation Factor Gene GFM1.Prevalent coordination of mitochondrial DNA transcription and initiation of replication with the cell cycleScreen for abnormal mitochondrial phenotypes in mouse embryonic stem cells identifies a model for succinyl-CoA ligase deficiency and mtDNA depletion.Deoxynucleoside stress exacerbates the phenotype of a mouse model of mitochondrial neurogastrointestinal encephalopathy.The GABA transaminase, ABAT, is essential for mitochondrial nucleoside metabolism.Mitochondrial Neurogastrointestinal Encephalomyopathy Caused by Thymidine Phosphorylase Enzyme Deficiency: From Pathogenesis to Emerging Therapeutic Options.Erythrocyte-mediated delivery of recombinant enzymes.Mitochondrial purine and pyrimidine metabolism and beyond.A Critical Balance: dNTPs and the Maintenance of Genome StabilityGene therapy using a liver-targeted AAV vector restores nucleoside and nucleotide homeostasis in a murine model of MNGIE.Long-Term Restoration of Thymidine Phosphorylase Function and Nucleoside Homeostasis Using Hematopoietic Gene Therapy in a Murine Model of Mitochondrial Neurogastrointestinal Encephalomyopathy.Regulation of Small Mitochondrial DNA Replicative Advantage by Ribonucleotide Reductase in Saccharomyces cerevisiae.Transgene expression of Drosophila melanogaster nucleoside kinase reverses mitochondrial thymidine kinase 2 deficiency.Administration of deoxyribonucleosides or inhibition of their catabolism as a pharmacological approach for mitochondrial DNA depletion syndrome.Preclinical Efficacy and Safety Evaluation of Hematopoietic Stem Cell Gene Therapy in a Mouse Model of MNGIE.Thymidine kinase 2 mutations in autosomal recessive progressive external ophthalmoplegia with multiple mitochondrial DNA deletions.Cardiomyocyte hypertrophy induced by Endonuclease G deficiency requires reactive oxygen radicals accumulation and is inhibitable by the micropeptide humanin.
P2860
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P2860
Limited dCTP availability accounts for mitochondrial DNA depletion in mitochondrial neurogastrointestinal encephalomyopathy (MNGIE)
description
2011 nî lūn-bûn
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2011 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2011 թվականի մարտին հրատարակված գիտական հոդված
@hy
2011年の論文
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2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
name
Limited dCTP availability acco ...... inal encephalomyopathy (MNGIE)
@ast
Limited dCTP availability acco ...... inal encephalomyopathy (MNGIE)
@en
Limited dCTP availability acco ...... inal encephalomyopathy (MNGIE)
@nl
type
label
Limited dCTP availability acco ...... inal encephalomyopathy (MNGIE)
@ast
Limited dCTP availability acco ...... inal encephalomyopathy (MNGIE)
@en
Limited dCTP availability acco ...... inal encephalomyopathy (MNGIE)
@nl
prefLabel
Limited dCTP availability acco ...... inal encephalomyopathy (MNGIE)
@ast
Limited dCTP availability acco ...... inal encephalomyopathy (MNGIE)
@en
Limited dCTP availability acco ...... inal encephalomyopathy (MNGIE)
@nl
P2860
P1433
P1476
Limited dCTP availability acco ...... inal encephalomyopathy (MNGIE)
@en
P2093
Antoni L Andreu
Javier Torres-Torronteras
P2860
P304
P356
10.1371/JOURNAL.PGEN.1002035
P407
P577
2011-03-31T00:00:00Z