FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2981056560> ?p ?o ?g. }

• W2981056560 endingPage "82" @default.
• W2981056560 startingPage "53" @default.
• W2981056560 abstract "The occurrence of 3-methylglutaconic aciduria (3-MGA) is a well understood phenomenon in leucine oxidation and ketogenesis disorders (primary 3-MGAs). In contrast, its genesis in non-canonical (secondary) 3-MGAs, a growing-up group of disorders encompassing more than a dozen of inherited metabolic diseases, is a mystery still remaining unresolved for three decades. To puzzle out this anthologic problem of metabolism, three clues were considered: (i) the variety of disorders suggests a common cellular target at the cross-road of metabolic and signaling pathways, (ii) the response to leucine loading test only discriminative for primary but not secondary 3-MGAs suggests these latter are disorders of extramitochondrial HMG-CoA metabolism as also attested by their failure to increase 3-hydroxyisovalerate, a mitochondrial metabolite accumulating only in primary 3-MGAs, (iii) the peroxisome is an extramitochondrial site possessing its own pool and displaying metabolism of HMG-CoA, suggesting its possible involvement in producing extramitochondrial 3-methylglutaconate (3-MG). Following these clues provides a unifying common basis to non-canonical 3-MGAs: constitutive mitochondrial dysfunction induces AMPK activation which, by inhibiting early steps in cholesterol and fatty acid syntheses, pipelines cytoplasmic acetyl-CoA to peroxisomes where a rise in HMG-CoA followed by local dehydration and hydrolysis may lead to 3-MGA yield. Additional contributors are considered, notably for 3-MGAs associated with hyperammonemia, and to a lesser extent in CLPB deficiency. Metabolic and signaling itineraries followed by the proposed scenario are essentially sketched, being provided with compelling evidence from the literature coming in their support." @default.
• W2981056560 created "2019-10-25" @default.
• W2981056560 creator A5026837342 @default.
• W2981056560 creator A5030960769 @default.
• W2981056560 creator A5032755271 @default.
• W2981056560 creator A5042648687 @default.
• W2981056560 creator A5065708547 @default.
• W2981056560 creator A5067146053 @default.
• W2981056560 date "2020-01-01" @default.
• W2981056560 modified "2023-10-14" @default.
• W2981056560 title "Mitochondrial dysfunction, AMPK activation and peroxisomal metabolism: A coherent scenario for non-canonical 3-methylglutaconic acidurias" @default.
• W2981056560 cites W1125737316 @default.
• W2981056560 cites W1485824567 @default.
• W2981056560 cites W1491296468 @default.
• W2981056560 cites W1509847058 @default.
• W2981056560 cites W1524194816 @default.
• W2981056560 cites W1528175130 @default.
• W2981056560 cites W1528807955 @default.
• W2981056560 cites W1544426283 @default.
• W2981056560 cites W1547467983 @default.
• W2981056560 cites W1550090553 @default.
• W2981056560 cites W1553155385 @default.
• W2981056560 cites W1559210758 @default.
• W2981056560 cites W1570483040 @default.
• W2981056560 cites W1582955199 @default.
• W2981056560 cites W1588691299 @default.
• W2981056560 cites W1591225500 @default.
• W2981056560 cites W1593455383 @default.
• W2981056560 cites W1598255749 @default.
• W2981056560 cites W1600980171 @default.
• W2981056560 cites W1618265444 @default.
• W2981056560 cites W16324672 @default.
• W2981056560 cites W1663324286 @default.
• W2981056560 cites W1750179163 @default.
• W2981056560 cites W1763491898 @default.
• W2981056560 cites W1765013321 @default.
• W2981056560 cites W1782438347 @default.
• W2981056560 cites W1787339412 @default.
• W2981056560 cites W1797227472 @default.
• W2981056560 cites W1801883537 @default.
• W2981056560 cites W1804257141 @default.
• W2981056560 cites W1820085648 @default.
• W2981056560 cites W1830411167 @default.
• W2981056560 cites W1841278993 @default.
• W2981056560 cites W1842204188 @default.
• W2981056560 cites W1853192652 @default.
• W2981056560 cites W1853579944 @default.
• W2981056560 cites W1912507042 @default.
• W2981056560 cites W1942537637 @default.
• W2981056560 cites W1945465158 @default.
• W2981056560 cites W1946982336 @default.
• W2981056560 cites W1953598538 @default.
• W2981056560 cites W1964261673 @default.
• W2981056560 cites W1965023277 @default.
• W2981056560 cites W1965098641 @default.
• W2981056560 cites W1966339676 @default.
• W2981056560 cites W1966706887 @default.
• W2981056560 cites W1967893549 @default.
• W2981056560 cites W1968555427 @default.
• W2981056560 cites W1969622294 @default.
• W2981056560 cites W1970002079 @default.
• W2981056560 cites W1970020574 @default.
• W2981056560 cites W1970607230 @default.
• W2981056560 cites W1970886233 @default.
• W2981056560 cites W1971428551 @default.
• W2981056560 cites W1972661072 @default.
• W2981056560 cites W1973554368 @default.
• W2981056560 cites W1973756521 @default.
• W2981056560 cites W1974358702 @default.
• W2981056560 cites W1975322691 @default.
• W2981056560 cites W1975582182 @default.
• W2981056560 cites W1975815179 @default.
• W2981056560 cites W1976081400 @default.
• W2981056560 cites W1976863495 @default.
• W2981056560 cites W1977252354 @default.
• W2981056560 cites W1977914031 @default.
• W2981056560 cites W1978058114 @default.
• W2981056560 cites W1978679812 @default.
• W2981056560 cites W1978747518 @default.
• W2981056560 cites W1979155967 @default.
• W2981056560 cites W1980142622 @default.
• W2981056560 cites W1980407361 @default.
• W2981056560 cites W1980636779 @default.
• W2981056560 cites W1981761696 @default.
• W2981056560 cites W1983382217 @default.
• W2981056560 cites W1984019957 @default.
• W2981056560 cites W1984816973 @default.
• W2981056560 cites W1985449116 @default.
• W2981056560 cites W1985703792 @default.
• W2981056560 cites W1986865985 @default.
• W2981056560 cites W1987601583 @default.
• W2981056560 cites W1988333880 @default.
• W2981056560 cites W1988601007 @default.
• W2981056560 cites W1988632024 @default.
• W2981056560 cites W1988894889 @default.
• W2981056560 cites W1989939332 @default.
• W2981056560 cites W1990153284 @default.
• W2981056560 cites W1991845210 @default.

• next