Glutarate Hydroxylation by the Carbon Starvation-Induced Protein D: A Computational Study into the Stereo- and Regioselectivities of the Reaction.
Han SB, Ali HS, Ali HS, de Visser SP.
Inorg Chem · journal article · DOI 10.1021/acs.inorgchem.0c03749 PUBLIKATIONSREGISTRET
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10 träffarHypoglycemia is not a defining feature of metabolic crisis in mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase deficiency: Further evidence of specific biochemical markers which may aid diagnosis.
Conlon TA, Fitzsimons PE, Borovickova I, Kirby F, Murphy S, Knerr I, Crushell E.
JIMD Rep · journal article; case reports; case-report · DOI 10.1002/jmd2.12146Ammonium accumulation and cell death in a rat 3D brain cell model of glutaric aciduria type I.
Jafari P, Braissant O, Zavadakova P, Henry H, Bonafé L, Ballhausen D.
PLoS One · research support, non-u.s. gov't; research-article; journal article · DOI 10.1371/journal.pone.0053735The sodium-dependent di- and tricarboxylate transporter, NaCT, is not responsible for the uptake of D-, L-2-hydroxyglutarate and 3-hydroxyglutarate into neurons.
Brauburger K, Burckhardt G, Burckhardt BC.
J Inherit Metab Dis · research support, non-u.s. gov't; research-article; journal article · DOI 10.1007/s10545-010-9268-23-Hydroxyglutaric acid is transported via the sodium-dependent dicarboxylate transporter NaDC3.
Stellmer F, Keyser B, Burckhardt BC, Koepsell H, Streichert T, Glatzel M, Jabs S, Thiem J, Herdering W, Koeller DM, Goodman SI, Lukacs Z, Ullrich K, Burckhardt G, Braulke T, Mühlhausen C.
J Mol Med (Berl) · research support, non-u.s. gov't; journal article · DOI 10.1007/s00109-007-0174-5Glutaric aciduria I: creatine supplementation restores creatinephosphate levels in mixed cortex cells from rat incubated with 3-hydroxyglutarate.
Das AM, Lücke T, Ullrich K.
Mol Genet Metab · journal article · DOI 10.1016/s1096-7192(02)00227-5Ca(2+) and Na(+) dependence of 3-hydroxyglutarate-induced excitotoxicity in primary neuronal cultures from chick embryo telencephalons.
Kölker S, Köhr G, Ahlemeyer B, Okun JG, Pawlak V, Hörster F, Mayatepek E, Krieglstein J, Hoffmann GF.
Pediatr Res · research support, non-u.s. gov't; journal article · DOI 10.1203/00006450-200208000-000113-Hydroxyglutarate excretion is increased in ketotic patients: implications for glutaryl-CoA dehydrogenase deficiency testing.
Pitt J, Carpenter K, Wilcken B, Boneh A.
J Inherit Metab Dis · journal article; case reports · DOI 10.1023/a:1015654608166Contribution of reactive oxygen species to 3-hydroxyglutarate neurotoxicity in primary neuronal cultures from chick embryo telencephalons.
Kölker S, Ahlemeyer B, Krieglstein J, Hoffmann GF.
Pediatr Res · research support, non-u.s. gov't; journal article · DOI 10.1203/00006450-200107000-00015Potentiation of 3-hydroxyglutarate neurotoxicity following induction of astrocytic iNOS in neonatal rat hippocampal cultures.
Kölker S, Ahlemeyer B, Hühne R, Mayatepek E, Krieglstein J, Hoffmann GF.
Eur J Neurosci · research support, non-u.s. gov't; journal article · DOI 10.1046/j.0953-816x.2001.01595.x