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Page 1
alpha-Ketoadipic Acid and alpha-Aminoadipic Acid Cause Disturbance of Glutamatergic Neurotransmission and Induction of Oxidative Stress In Vitro in Brain of Adolescent Rats.
da Silva JC, Amaral AU, Cecatto C, Wajner A, Dos Santos Godoy K, Ribeiro RT, de Mello Gonçalves A, Zanatta Â, da Rosa MS, Loureiro SO, Vargas CR, Leipnitz G, de Souza DOG, Wajner M. da Silva JC, et al. Neurotox Res. 2017 Aug;32(2):276-290. doi: 10.1007/s12640-017-9735-8. Epub 2017 Apr 20. Neurotox Res. 2017. PMID: 28429309
Genetic basis of alpha-aminoadipic and alpha-ketoadipic aciduria.
Hagen J, te Brinke H, Wanders RJ, Knegt AC, Oussoren E, Hoogeboom AJ, Ruijter GJ, Becker D, Schwab KO, Franke I, Duran M, Waterham HR, Sass JO, Houten SM. Hagen J, et al. J Inherit Metab Dis. 2015 Sep;38(5):873-9. doi: 10.1007/s10545-015-9841-9. Epub 2015 Apr 10. J Inherit Metab Dis. 2015. PMID: 25860818
Our results confirm that alpha-aminoadipic and alpha-ketoadipic aciduria is caused by mutations in DHTKD1, and further establish that DHTKD1 encodes the E1 subunit of the alpha-ketoadipic acid dehydrogenase complex....
Our results confirm that alpha-aminoadipic and alpha-ketoadipic aciduria is caused by mutations in DHTKD1, and further establish that DHTKD1 …
Inactivation of alpha-ketoglutarate dehydrogenase during oxidative decarboxylation of alpha-ketoadipic acid.
Bunik VI, Pavlova OG. Bunik VI, et al. FEBS Lett. 1993 May 24;323(1-2):166-70. doi: 10.1016/0014-5793(93)81472-c. FEBS Lett. 1993. PMID: 8495733 Free article.
alpha-Ketoglutarate dehydrogenase was inactivated irreversibly and completely during oxidation of alpha-ketoadipic acid. The inactivation was revealed both in the model system with ferricyanide and in the overall reaction catalyzed by the alpha-ketoglutarate …
alpha-Ketoglutarate dehydrogenase was inactivated irreversibly and completely during oxidation of alpha-ketoadipic acid …
Identification of N-acetyl-alpha-aminoadipic acid in the urine of a patient with alpha-aminoadipic and alpha-ketoadipic aciduria.
Takechi T, Okada T, Wakiguchi H, Morita H, Kurashige T, Sugahara K, Kodama H. Takechi T, et al. J Inherit Metab Dis. 1993;16(1):119-26. doi: 10.1007/BF00711325. J Inherit Metab Dis. 1993. PMID: 8487492
The concentrations of alpha-aminoadipic acid and N-acetyl-alpha-aminoadipic acid in the urine of a patient with alpha-aminoadipic aciduria were 376.9 nmol/mg creatinine and 18.1 nmol/mg creatinine, respectively. We also detected alpha-ketoadipic acid in the u …
The concentrations of alpha-aminoadipic acid and N-acetyl-alpha-aminoadipic acid in the urine of a patient with alpha-aminoadipic aciduria w …
Sudden death of a 2-year-old child due to alpha-ketoadipic aciduria.
Kondou H, Ichioka H, Akasaka Y, Kondo H, Ikegaya H. Kondou H, et al. Forensic Sci Med Pathol. 2022 Dec;18(4):474-477. doi: 10.1007/s12024-022-00483-4. Epub 2022 Jun 1. Forensic Sci Med Pathol. 2022. PMID: 35648288
Its pathophysiology is reduced alpha-ketoadipic acid dehydrogenase activity, and that makes it difficult to metabolize lysine and tryptophan. ...No obvious lesions could be identified except for a marked fatty liver. Urinalysis showed elevated excretion of …
Its pathophysiology is reduced alpha-ketoadipic acid dehydrogenase activity, and that makes it difficult to metabolize …
Metabolism of alpha-aminoadipic and alpha-ketoadipic acids: studies using rat and beef liver, and human leukocytes.
Wilson RW, Wilson CM, Higgins JV. Wilson RW, et al. Clin Chim Acta. 1976 Jun 1;69(2):323-32. doi: 10.1016/0009-8981(76)90512-x. Clin Chim Acta. 1976. PMID: 1277562
The reported studies have shown that alpha-DL-amino [1-14C]adipic acid is metabolized to radioactive alpha-ketoadipic acid and then to 14CO2 in rat and beef liver homogenates. ...
The reported studies have shown that alpha-DL-amino [1-14C]adipic acid is metabolized to radioactive alpha-ketoadipic acid …
Alpha-ketoadipic aciduria, a new inborn error of lysine metabolism; biochemical studies.
Przyrembel H, Bachmann D, Lombeck I, Becker K, Wendel U, Wadman SK, Bremer HJ. Przyrembel H, et al. Clin Chim Acta. 1975 Feb 8;58(3):257-69. doi: 10.1016/0009-8981(75)90445-3. Clin Chim Acta. 1975. PMID: 1112064
Investigation of a psychomotorically retarded girl showed excretion of abnormal amounts of alpha-ketoadipic acid, alpha-hydroxyadipic acid, alpha-aminoadipic acid, 1,2-butenedicarboxylic acid and elevation of plasma alpha-aminoadipic acid levels. ...Degradati …
Investigation of a psychomotorically retarded girl showed excretion of abnormal amounts of alpha-ketoadipic acid, alpha …
Engineering of phenylalanine dehydrogenase from Thermoactinomyces intermedius for the production of a novel homoglutamate.
Tariq M, Israr M, Raza M, Ahmad B, Azizullah A, Ur Rehman S, Faheem M, Sun X, Yuan Q. Tariq M, et al. PLoS One. 2022 Mar 30;17(3):e0263784. doi: 10.1371/journal.pone.0263784. eCollection 2022. PLoS One. 2022. PMID: 35353822 Free PMC article.
Based on computational insight into the structure, we proposed the site-specific directed mutagenesis of the two important residues of PDH through docking simulations by AutoDock Vina which elucidated the binding mode of PDH with alpha-Ketoadipic acid and lig …
Based on computational insight into the structure, we proposed the site-specific directed mutagenesis of the two important residues of PDH t …
50 results