FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2105342146> ?p ?o ?g. }

• W2105342146 endingPage "H1297" @default.
• W2105342146 startingPage "H1289" @default.
• W2105342146 abstract "Mitochondrial very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is associated with severe hypoglycemia, cardiac dysfunction, and sudden death in neonates and children. Sudden death is common, but the underlying mechanisms are not fully understood. We report on a mouse model of VLCAD deficiency with a phenotype induced by the stresses of fasting and cold, which includes hypoglycemia, hypothermia, and severe bradycardia. The administration of glucose did not rescue the mice under stress conditions, but rewarming alone consistently led to heart rate recovery. Brown adipose tissue (BAT) from the VLCAD −/− mice showed elevated levels of the uncoupling protein isoforms and peroxisome proliferator-activated receptor-α. Biochemical assessment of the VLCAD −/− mice BAT showed increased oxygen consumption, attributed to uncoupled respiration in the absence of stress. ADP-stimulated respiration was 23.05 (SD 4.17) and 68.24 (SD 6.3) nmol O 2 ·min −1 ·mg mitochondrial protein −1 for VLCAD +/+ and VLCAD −/− mice, respectively ( P < 0.001), and carbonyl cyanide p-trifluoromethoxyphenylhydrazone-stimulated respiration was 35.9 (SD 3.6) and 49.3 (SD 9) nmol O 2 ·min −1 ·mg mitochondrial protein −1 for VLCAD +/+ and VLCAD −/− mice, respectively ( P < 0.20), but these rates were insufficient to protect them in the cold. We conclude that disturbed mitochondrial bioenergetics in BAT is a critical contributing factor for the cold sensitivity in VLCAD deficiency. Our observations provide insights into the possible mechanisms of stress-induced death in human newborns with abnormal fat metabolism and elucidate targeting of specific substrates for particular metabolic needs." @default.
• W2105342146 created "2016-06-24" @default.
• W2105342146 creator A5024945517 @default.
• W2105342146 creator A5029548649 @default.
• W2105342146 creator A5033653473 @default.
• W2105342146 creator A5047720255 @default.
• W2105342146 creator A5053385911 @default.
• W2105342146 creator A5063730520 @default.
• W2105342146 creator A5065378322 @default.
• W2105342146 creator A5081875295 @default.
• W2105342146 creator A5083810391 @default.
• W2105342146 date "2006-03-01" @default.
• W2105342146 modified "2023-09-30" @default.
• W2105342146 title "Abnormal mitochondrial bioenergetics and heart rate dysfunction in mice lacking very-long-chain acyl-CoA dehydrogenase" @default.
• W2105342146 cites W1895143475 @default.
• W2105342146 cites W1900866157 @default.
• W2105342146 cites W1970919740 @default.
• W2105342146 cites W1972191708 @default.
• W2105342146 cites W1994212260 @default.
• W2105342146 cites W1998249792 @default.
• W2105342146 cites W2000913580 @default.
• W2105342146 cites W2003762672 @default.
• W2105342146 cites W2004235323 @default.
• W2105342146 cites W2008632158 @default.
• W2105342146 cites W2008965061 @default.
• W2105342146 cites W2010438605 @default.
• W2105342146 cites W2018912156 @default.
• W2105342146 cites W2030201364 @default.
• W2105342146 cites W2034420020 @default.
• W2105342146 cites W2056461086 @default.
• W2105342146 cites W2060176936 @default.
• W2105342146 cites W2062073832 @default.
• W2105342146 cites W2068897440 @default.
• W2105342146 cites W2079477578 @default.
• W2105342146 cites W2081520162 @default.
• W2105342146 cites W2089019519 @default.
• W2105342146 cites W2100463267 @default.
• W2105342146 cites W2101527214 @default.
• W2105342146 cites W2102326852 @default.
• W2105342146 cites W2104385322 @default.
• W2105342146 cites W2109835425 @default.
• W2105342146 cites W2117047583 @default.
• W2105342146 cites W2117820310 @default.
• W2105342146 cites W2155793022 @default.
• W2105342146 cites W2166788192 @default.
• W2105342146 cites W4242169888 @default.
• W2105342146 doi "https://doi.org/10.1152/ajpheart.00811.2005" @default.
• W2105342146 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/16199475" @default.
• W2105342146 hasPublicationYear "2006" @default.
• W2105342146 type Work @default.
• W2105342146 sameAs 2105342146 @default.
• W2105342146 citedByCount "47" @default.
• W2105342146 countsByYear W21053421462012 @default.
• W2105342146 countsByYear W21053421462013 @default.
• W2105342146 countsByYear W21053421462014 @default.
• W2105342146 countsByYear W21053421462015 @default.
• W2105342146 countsByYear W21053421462016 @default.
• W2105342146 countsByYear W21053421462017 @default.
• W2105342146 countsByYear W21053421462018 @default.
• W2105342146 countsByYear W21053421462019 @default.
• W2105342146 countsByYear W21053421462020 @default.
• W2105342146 countsByYear W21053421462022 @default.
• W2105342146 countsByYear W21053421462023 @default.
• W2105342146 crossrefType "journal-article" @default.
• W2105342146 hasAuthorship W2105342146A5024945517 @default.
• W2105342146 hasAuthorship W2105342146A5029548649 @default.
• W2105342146 hasAuthorship W2105342146A5033653473 @default.
• W2105342146 hasAuthorship W2105342146A5047720255 @default.
• W2105342146 hasAuthorship W2105342146A5053385911 @default.
• W2105342146 hasAuthorship W2105342146A5063730520 @default.
• W2105342146 hasAuthorship W2105342146A5065378322 @default.
• W2105342146 hasAuthorship W2105342146A5081875295 @default.
• W2105342146 hasAuthorship W2105342146A5083810391 @default.
• W2105342146 hasConcept C100206155 @default.
• W2105342146 hasConcept C111133373 @default.
• W2105342146 hasConcept C126322002 @default.
• W2105342146 hasConcept C134018914 @default.
• W2105342146 hasConcept C171089720 @default.
• W2105342146 hasConcept C181199279 @default.
• W2105342146 hasConcept C182215343 @default.
• W2105342146 hasConcept C2777390192 @default.
• W2105342146 hasConcept C2779306644 @default.
• W2105342146 hasConcept C2780642333 @default.
• W2105342146 hasConcept C2780668416 @default.
• W2105342146 hasConcept C28859421 @default.
• W2105342146 hasConcept C55493867 @default.
• W2105342146 hasConcept C59822182 @default.
• W2105342146 hasConcept C71924100 @default.
• W2105342146 hasConcept C86803240 @default.
• W2105342146 hasConceptScore W2105342146C100206155 @default.
• W2105342146 hasConceptScore W2105342146C111133373 @default.
• W2105342146 hasConceptScore W2105342146C126322002 @default.
• W2105342146 hasConceptScore W2105342146C134018914 @default.
• W2105342146 hasConceptScore W2105342146C171089720 @default.
• W2105342146 hasConceptScore W2105342146C181199279 @default.
• W2105342146 hasConceptScore W2105342146C182215343 @default.
• W2105342146 hasConceptScore W2105342146C2777390192 @default.
• W2105342146 hasConceptScore W2105342146C2779306644 @default.

• next