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Year Number of Results
1980 1
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1990 2
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1993 2
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1998 6
1999 2
2000 4
2001 5
2002 4
2003 6
2004 8
2005 3
2006 3
2007 4
2008 3
2009 3
2010 1
2011 2
2012 3
2013 5
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144 results

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Page 1
The Critical Role of the Branched Chain Amino Acids (BCAAs) Catabolism-Regulating Enzymes, Branched-Chain Aminotransferase (BCAT) and Branched-Chain alpha-Keto Acid Dehydrogenase (BCKD), in Human Pathophysiology.
Dimou A, Tsimihodimos V, Bairaktari E. Dimou A, et al. Int J Mol Sci. 2022 Apr 5;23(7):4022. doi: 10.3390/ijms23074022. Int J Mol Sci. 2022. PMID: 35409380 Free PMC article. Review.
However, downregulated/upregulated plasma BCAAs and their defective catabolism in various tissues, mainly due to altered enzymatic activity of the first two enzymes in their catabolic pathway, BCAA aminotransferase (BCAT) and branched-chain alpha-keto acid dehydrogenase (BCKD …
However, downregulated/upregulated plasma BCAAs and their defective catabolism in various tissues, mainly due to altered enzymatic activity …
Maple Syrup Urine Disease.
Strauss KA, Puffenberger EG, Carson VJ. Strauss KA, et al. 2006 Jan 30 [updated 2020 Apr 23]. In: Adam MP, Bick S, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2026. 2006 Jan 30 [updated 2020 Apr 23]. In: Adam MP, Bick S, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2026. PMID: 20301495 Free Books & Documents. Review.
Treatment of maple syrup urine disease: Benefits, risks, and challenges of liver transplantation.
Deon M, Guerreiro G, Girardi J, Ribas G, Vargas CR. Deon M, et al. Int J Dev Neurosci. 2023 Oct;83(6):489-504. doi: 10.1002/jdn.10283. Epub 2023 Jun 20. Int J Dev Neurosci. 2023. PMID: 37340513 Review.
Maple syrup urine disease (MSUD) is caused by a deficiency in the activity of the branched-chain alpha-ketoacid dehydrogenase (BCKD) complex, promoting the accumulation of the branched-chain amino acids (BCAA) leucine, isoleucine, and valine, as well as their respective al …
Maple syrup urine disease (MSUD) is caused by a deficiency in the activity of the branched-chain alpha-ketoacid dehydrogenase (BCKD) …
Branched-chain amino acids in health and disease: metabolism, alterations in blood plasma, and as supplements.
Holeček M. Holeček M. Nutr Metab (Lond). 2018 May 3;15:33. doi: 10.1186/s12986-018-0271-1. eCollection 2018. Nutr Metab (Lond). 2018. PMID: 29755574 Free PMC article. Review.
Increased BCKD activity is responsible for enhanced oxidation of BCAA in chronic renal failure, trauma, burn, sepsis, cancer, phenylbutyrate-treated subjects, and during exercise. Decreased BCKD activity is the main cause of increased BCAA levels and BCKAs in maple …
Increased BCKD activity is responsible for enhanced oxidation of BCAA in chronic renal failure, trauma, burn, sepsis, cancer, phenylb …
Research experimental design for the construction and identification of the pGEX-BCKD-E4A recombinant point-mutant plasmid.
Zhou T, Wei H, Wang J. Zhou T, et al. PLoS One. 2023 Feb 24;18(2):e0279431. doi: 10.1371/journal.pone.0279431. eCollection 2023. PLoS One. 2023. PMID: 36827343 Free PMC article.
The students first constructed the recombinant plasmid pGEX-BCKD using the vector plasmid pGEX-4T1 and the target gene fragment BCKD provided by the laboratory and used this as a template to construct the pGEX-BCKD-E4A point mutation plasmid by the overlap ex …
The students first constructed the recombinant plasmid pGEX-BCKD using the vector plasmid pGEX-4T1 and the target gene fragment BC …
The mitochondrial BCKD complex interacts with hepatic apolipoprotein E in cultured cells in vitro and mouse livers in vivo.
Rueter J, Rimbach G, Treitz C, Schloesser A, Lüersen K, Tholey A, Huebbe P. Rueter J, et al. Cell Mol Life Sci. 2023 Feb 7;80(3):59. doi: 10.1007/s00018-023-04706-x. Cell Mol Life Sci. 2023. PMID: 36749362 Free PMC article.
Proteomic analysis of coimmunoprecipitation products from APOE mouse liver extracts revealed 28 APOE-interacting candidate proteins, including branched-chain alpha-keto acid dehydrogenase (BCKD) complex subunit alpha (BCKDHA) and voltage-dependent anion-selective channel 1 …
Proteomic analysis of coimmunoprecipitation products from APOE mouse liver extracts revealed 28 APOE-interacting candidate proteins, includi …
PPM1K mediates metabolic disorder of branched-chain amino acid and regulates cerebral ischemia-reperfusion injury by activating ferroptosis in neurons.
Li T, Zhao L, Li Y, Dang M, Lu J, Lu Z, Huang Q, Yang Y, Feng Y, Wang X, Jian Y, Wang H, Guo Y, Zhang L, Jiang Y, Fan S, Wu S, Fan H, Kuang F, Zhang G. Li T, et al. Cell Death Dis. 2023 Sep 26;14(9):634. doi: 10.1038/s41419-023-06135-x. Cell Death Dis. 2023. PMID: 37752100 Free PMC article.
We established the I/R model in mice and used BT2, a highly specific BCAA dehydrogenase (BCKD) kinase inhibitor to promote BCAA metabolism. It was further verified by lentivirus knocking down PPM1K in neurons. We found that BCAA levels were elevated after I/R injury due to …
We established the I/R model in mice and used BT2, a highly specific BCAA dehydrogenase (BCKD) kinase inhibitor to promote BCAA metab …
Comparative metabolism and structure of BCKD-E2 in primary biliary cirrhosis.
Turchany JM, Leung PS, Iwayama T, Jefferson DM, Ishida J, Yamaguchi M, Munoz S, Danner DJ, Dickson ER, Gershwin ME. Turchany JM, et al. J Autoimmun. 1993 Aug;6(4):459-66. doi: 10.1006/jaut.1993.1038. J Autoimmun. 1993. PMID: 8216688
The two major autoantigens are the E2 subunits of pyruvate dehydrogenase and branched-chain ketoacid dehydrogenase (BCKD). Interestingly, one of these complexes, BCKD-E2, is already well known to clinical medicine based on its association with genetic mutations in m …
The two major autoantigens are the E2 subunits of pyruvate dehydrogenase and branched-chain ketoacid dehydrogenase (BCKD). Interestin …
Controlled overexpression of BCKD kinase expression: metabolic engineering applied to BCAA metabolism in a mammalian system.
Doering CB, Williams IR, Danner DJ. Doering CB, et al. Metab Eng. 2000 Oct;2(4):349-56. doi: 10.1006/mben.2000.0164. Metab Eng. 2000. PMID: 11120646
In this study, doxycycline treatment leads to a 10-fold increase in BCKD kinase protein. The transgene-generated kinase is rapidly incorporated within mitochondria and functions correctly to inactivate the BCKD complex. The maximum reduction in basal BCKD act …
In this study, doxycycline treatment leads to a 10-fold increase in BCKD kinase protein. The transgene-generated kinase is rapidly in …
Mutations in BCKD-kinase lead to a potentially treatable form of autism with epilepsy.
Novarino G, El-Fishawy P, Kayserili H, Meguid NA, Scott EM, Schroth J, Silhavy JL, Kara M, Khalil RO, Ben-Omran T, Ercan-Sencicek AG, Hashish AF, Sanders SJ, Gupta AR, Hashem HS, Matern D, Gabriel S, Sweetman L, Rahimi Y, Harris RA, State MW, Gleeson JG. Novarino G, et al. Science. 2012 Oct 19;338(6105):394-7. doi: 10.1126/science.1224631. Epub 2012 Sep 6. Science. 2012. PMID: 22956686 Free PMC article.
144 results