
NCBI . NLM . NIH . GOV {
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Title[redir]:
Mtu1-Mediated Thiouridine Formation of Mitochondrial tRNAs Is Required for Mitochondrial Translation and Is Involved in Reversible Infantile Liver Injury - PMC
Description:
Reversible infantile liver failure (RILF) is a unique heritable liver disease characterized by acute liver failure followed by spontaneous recovery at an early stage of life. Genetic mutations in MTU1 have been identified in RILF patients. MTU1 is a ...
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Keywords {🔍}
mice, mitochondrial, mtu, mtulko, fig, liver, translation, levels, hepatocytes, lko, doi, google, scholar, pubmed, rilf, flox, pmc, modification, article, mtudeficient, mtuflox, livers, modifications, data, free, patients, knockout, exhibited, deficiency, stress, tif, weekold, relative, control, mutations, oxidative, results, representative, respiratory, mttrnas, shown, injury, molecular, dysfunction, mouse, protein, examined, trnas, gene, genes,
Topics {✒️}
generated liver-specific mtu1-deficient muscle-specific mtu1-deificient mice hepatocyte-specific mtu1 knockout generated liver-specific knockout liver-bone endocrine relay von kleist-retzow jc mt-trna-modifying enzymes pmc beta search primerscript rt-pcr kit glucose-6-phosphatase catalytic subunit cdk5rap1-mediated 2-methylthio modification high-density lipoprotein cholesterol mitochondrial mt-tf variant low-density lipoprotein cholesterol trna-modifying enzymes mitochondrial aspartyl-trna synthetase life-threatening condition characterized blood vessel networks mtu1-mediated 2-thiouridine formation [google scholar] 5 adult mtu1-deficient mice mtu1-mediated mitochondrial translation induced liver injury mtu1-deficient primary hepatocytes selected metabolism-related genes infantile reversible cytochrome mtu1-deficient hepatocytes compared mtu1-mediated s2 modification mtdna-encoded mitochondrial genes oxidative stress-related metabolites aberrant structures magnified liver injury characterized constitutive knockout mice respiratory chain deficiency mt-trnalys remained unchanged blue-native page developmental stage-matched tissues fully modified mt-trnas data availability statement exhibited normal translation catastrophic liver failure dars2 knockout mice liver injury occurs publisher site pdf severe mitochondrial dysfunction strain-specific differences mt-trnalys contained τm5s2u bacterial trnagln lacking 12% sds-page gels mtu1-deficient liver
Questions {❓}
- Why is Mtu1-mediated s2 modification crucial for mitochondrial translation?
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- Google Tag Manager
Libraries {📚}
- jQuery
- jQuery module (jquery-3.6.0)
- Zoom.js
Emails and Hosting {✉️}
Mail Servers:
- nihcesxway.hub.nih.gov
- nihcesxway2.hub.nih.gov
- nihcesxway3.hub.nih.gov
- nihcesxway4.hub.nih.gov
- nihcesxway5.hub.nih.gov
Name Servers:
- dns1-ncbi.ncbi.nlm.nih.gov
- dns2-ncbi.ncbi.nlm.nih.gov
- lhcns1.nlm.nih.gov
- lhcns2.nlm.nih.gov
CDN Services {📦}
- Ncbi