|1.||Wajner, Moacir: 4 articles (03/2008 - 06/2005)|
|2.||Lynch, Christopher J: 3 articles (12/2014 - 12/2007)|
|3.||Sitta, Angela: 3 articles (03/2008 - 12/2006)|
|4.||Vargas, Carmen R: 3 articles (03/2008 - 12/2006)|
|5.||Deon, Marion: 3 articles (03/2008 - 12/2006)|
|6.||Dutra-Filho, Carlos S: 3 articles (03/2008 - 12/2006)|
|7.||Barschak, Alethéa G: 3 articles (03/2008 - 12/2006)|
|8.||Shimomura, Yoshiharu: 2 articles (10/2009 - 03/2004)|
|9.||Sato, Yuzo: 2 articles (10/2009 - 03/2004)|
|10.||Nagasaki, Masaru: 2 articles (10/2009 - 03/2004)|
|1.||Maple Syrup Urine Disease
02/01/1989 - "The clinical, pathological and biochemical findings of a study of 30 Poll Hereford, Hereford, Poll Hereford cross or Hereford cross calves affected with branched chain ketoacid dehydrogenase (BCKAD) complex deficiency or maple syrup urine disease (MSUD) are presented. "
09/15/2014 - "Although deficiency of the branched-chain ketoacid dehydrogenase (BCKDC) and associated elevations in the BCAAs and their ketoacids have been recognized as the cause of maple syrup urine disease (MSUD) for decades, treatment options for this disorder have been limited to dietary interventions. "
10/01/2013 - "Maple syrup urine disease (MSUD) is predominantly caused by mutations in the BCKDHA, BCKDHB and DBT genes, which encode for the E1alpha, E1beta and E2 subunits of the branched-chain alpha-keto acid dehydrogenase complex, respectively. "
01/01/2013 - "Maple syrup urine disease (MSUD) is an inherited disorder caused by a deficiency of the mitochondrial branched-chain keto acid dehydrogenase complex. "
03/01/2011 - "Individuals with maple syrup urine disease (MSUD) have an inherited metabolic disorder resulting in a deficiency in the branched-chain keto-acid dehydrogenase complex. "
01/01/2001 - "The aim of the present study was to investigate changes in the activity of branched-chain alpha-keto acid dehydrogenase (BCKAD) in skeletal muscle and the heart during brief and prolonged starvation. "
03/01/2002 - "Activation of the promoters of Arabidopsis genes for the branched-chain alpha-keto acid dehydrogenase complex in transgenic tobacco BY-2 cells under sugar starvation."
01/01/2001 - "Effect of starvation on branched-chain alpha-keto acid dehydrogenase activity in rat heart and skeletal muscle."
06/01/1999 - "The effects of acute exercise and starvation on hepatic branched-chain alpha-keto acid dehydrogenase (BCKDH) complex activity were examined in female rats fed high (30%)- or low (8%)-protein diets. "
06/01/1999 - "Hepatic branched-chain alpha-keto acid dehydrogenase complex in female rats: activation by exercise and starvation."
|3.||Biliary Liver Cirrhosis (Primary Biliary Cirrhosis)
01/01/1999 - "Detection of anti-branched chain 2-oxo acid dehydrogenase complex (BCOADC)-E2 antibody in primary biliary cirrhosis by ELISA using recombinant fusion protein."
01/01/1989 - "Regulation of the alpha-keto acid dehydrogenase complexes and their involvement in primary biliary cirrhosis."
06/01/2001 - "Autoantibodies of sera from patients with primary biliary cirrhosis recognize the alpha subunit of the decarboxylase component of human branched-chain 2-oxo acid dehydrogenase complex."
05/25/1989 - "Serum samples from 89 of the 93 patients with primary biliary cirrhosis reacted with either the pyruvate dehydrogenase-E2 or the branched-chain alpha-keto acid dehydrogenase protein. "
07/01/1993 - "Primary biliary cirrhosis (PBC) is characterized by lymphoid infiltrates in the portal tracts of the liver and the occurrence of antimitochondrial autoantibodies in serum directed against components of the pyruvate dehydrogenase complex and the other alpha-keto acid dehydrogenase complexes. "
04/01/1998 - "Stimulation of rat-liver branched-chain alpha-keto acid dehydrogenase activity by chronic metabolic acidosis."
06/01/1992 - "These changes were accompanied by a 104% increase in liver branched-chain ketoacid dehydrogenase (BCKAD) activity in rats with acidosis, similar to previously documented increases in skeletal muscle BCKAD activity caused by acidosis. "
01/01/1992 - "These changes were accompanied by a 104% increase in liver branched-chain ketoacid dehydrogenase (BCKAD) activity in rats with acidosis, similar to previously documented increases in skeletal muscle BCKAD activity caused by acidosis. "
06/01/1987 - "We conclude that acidosis affects the regulation of BCAA metabolism by enhancing flux through the transaminase and by directly stimulating oxidative catabolism through activation of branched-chain alpha-keto acid dehydrogenase."
12/01/1987 - "Since we have found that metabolic acidosis, by itself, stimulates muscle branched-chain, ketoacid dehydrogenase activity, another group of CRF and control rats was given NaHCO3 which corrected the acidosis, but not the azotemia. "
12/01/2014 - "Research on the role of individual and model-dependent differences in BCAA metabolism is needed, as several genes (BCKDHA, PPM1K, IVD and KLF15) have been designated as candidate genes for obesity and/or T2DM in humans, and distinct phenotypes of tissue-specific branched chain ketoacid dehydrogenase complex activity have been detected in animal models of obesity and T2DM. "
05/01/2014 - "However it is unclear how obesity affects alloisoleucine, a BCAA and pathognomonic marker of branched-chain keto acid dehydrogenase complex (BCKDC) disorders. "
12/01/2007 - "To explore loss of catabolic capacity as a potential contributor to the obesity-related rises in BCAAs, we assessed the first two enzymatic steps, catalyzed by mitochondrial branched chain amino acid aminotransferase (BCATm) or the branched chain alpha-keto acid dehydrogenase (BCKD E1alpha subunit) complex, in two rodent models of obesity (ob/ob mice and Zucker rats) and after surgical weight loss intervention in humans. "
|1.||Branched-Chain Amino Acids
|4.||Proteasome Endopeptidase Complex (Proteasome)
|7.||Pyruvic Acid (Pyruvate)
|10.||3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) (Alpha-Keto Acid Dehydrogenase)
|1.||Protein-Restricted Diet (Diet, Protein Restricted)
|2.||Enteral Nutrition (Feeding, Tube)