Results 11 to 20 of about 177,283 (188)

Production and characterization of murine models of classic and intermediate maple syrup urine disease [PDF]

BMC Medical Genetics, 2006
Background Maple Syrup Urine Disease (MSUD) is an inborn error of metabolism caused by a deficiency of branched-chain keto acid dehydrogenase. MSUD has several clinical phenotypes depending on the degree of enzyme deficiency.
Watkins Simon, Ferguson Carolyn, Skvorak Kristen, Homanics Gregg E, Paul Harbhajan S   +4 more
doaj   +2 more sources

Role of relaxation time scale in noisy signal transduction [PDF]

, 2014
Intracellular fluctuations, mainly triggered by gene expression, are an inevitable phenomenon observed in living cells. It influences generation of phenotypic diversity in genetically identical cells.
Banik, Suman K., Chaudhury, Pinaki, Maity, Alok Kumar   +2 more
core   +8 more sources

Branched-Chain Amino Acid Negatively Regulates KLF15 Expression via PI3K-AKT Pathway. [PDF]

, 2017
Recent studies have linked branched-chain amino acid (BCAA) with numerous metabolic diseases. However, the molecular basis of BCAA's roles in metabolic regulation remains to be established.
Dong, Weibing, Liu, Yunxia, Shao, Jing, Sun, Haipeng, Wang, Yibin, Zhou, Meiyi   +5 more
core   +2 more sources

Pancreatic hormones and amino acid levels following liver transplantation [PDF]

, 1987
Glucose intolerance, hyperinsulinemia, peripheral insulin resistance and hyperglucagonemia are common in patients with advanced liver disease. These abnormalities in the plasma levels of the pancreatic hormones, insulin and glucagon have been thought to ...
Chisholm, Collins, Collins, Conn, Creutzfeldt, Dudley, Eriksson, Fath, Fischer, Hahn, Hamish, Johnston, Kam, Kenny, Limberg, Marchesini, Marchesini, Marchesini, Marchesini, Megysi, Morgan, Muller, Nielsen, Pearly, Reilly, Robertson, Schander, Sherwin, Sherwin, Soeters, Starzl, Starzl, Trinder   +32 more
core   +1 more source

Studies on the production of branched-chain alcohols in engineered Ralstonia eutropha [PDF]

, 2012
Wild-type Ralstonia eutropha H16 produces polyhydroxybutyrate (PHB) as an intracellular carbon storage material during nutrient stress in the presence of excess carbon.
A Baez, A Ishizaki, A Pohlmann, A Slutzker, A Steinbüchel, A Steinbüchel, A Steinbüchel, Anthony J. Sinskey, B Blombach, B Bowien, BH Rehm, C Larroy, CF Budde, CF Budde, Christopher J. Brigham, CJ Brigham, Claudia S. Gai, D Jendrossek, D Leyval, DB Karr, DM Chipman, DM Chipman, DR Nielsen, DR Nielsen, DS Gogerty, E Schwartz, EA Savrasova, GM York, GM York, H Li, J Plassmeier, J Quandt, J Sheehan, JA McCourt, JC McGowan, Jingnan Lu, JJ Minty, K Kurosawa, K Vollherbst-Schneck, KM Smith, LA Hazelwood, LR Jarboe, M Plaza de la, M Potter, M Vyazmensky, ME Kovach, MR Connor, MR Connor, N Gollop, O Lenz, PF Palencia de, R Simon, S Atsumi, S Atsumi, S Atsumi, S Atsumi, S Engel, S Khanna, S Mendel, S Slater, SH Baer, T Fukui, T Zor, V Macho, WW Westerfield, Y Yan, YH Yang, ZQ Zhang   +67 more
core   +1 more source

Norvaline is accumulated after a down-shift of oxygen in [PDF]

, 2008
Background Norvaline is an unusual non-proteinogenic branched-chain amino acid which has been of interest especially during the early enzymological studies on regulatory mutants of the branched-chain amino acid pathway in Serratia marcescens.
Bernhardt Jörg, Liedert Christina, Falschlehner Christina, Soini Jaakko, Vuoristo Jussi, Neubauer Peter   +5 more
core   +1 more source

Branched chain aldehydes: production and breakdown pathways and relevance for flavour in foods [PDF]

, 2009
Branched aldehydes, such as 2-methyl propanal and 2- and 3-methyl butanal, are important flavour compounds in many food products, both fermented and non-fermented (heat-treated) products. The production and degradation of these aldehydes from amino acids
Engels, W.J.M., Smit, B.A., Smit, G.
core   +2 more sources

BCKDH: the missing link in apicomplexan mitochondrial metabolism is required for full virulence of Toxoplasma gondii and Plasmodium berghei [PDF]

, 2014
While the apicomplexan parasites Plasmodium falciparum and Toxoplasma gondii are thought to primarily depend on glycolysis for ATP synthesis, recent studies have shown that they can fully catabolize glucose in a canonical TCA cycle.
Barrett, Michael P., Billker, Oliver, Creek, Darren J., Limenitakis, Julien, Macrae, James I., McConville, Malcolm J., Modrzynska, Katarzyna K., Oppenheim, Rebecca D., Pino, Paco, Polonais, Valerie, Seeber, Frank, Soldati-Favre, Dominique   +11 more
core   +1 more source

Production of d-Branched-Chain Amino Acids by Lactic Acid Bacteria Carrying Homologs to Isoleucine 2-Epimerase of Lactobacillus buchneri

Frontiers in Microbiology, 2018
Isoleucine 2-epimerase (ILEP) is a novel branched-chain amino acid racemase isolated from Lactobacillus buchneri. In this study, we examined production of free d-branched-chain amino acids such as d-valine, d-leucine, and d-allo-isoleucine, using lactic ...
Yuta Mutaguchi, Kano Kasuga, Ikuo Kojima
doaj   +1 more source

Differences in gene expression of enzymes involved in branched-chain amino acid metabolism of abdominal subcutaneous adipose tissue between pregnant women with and without PCOS

Taiwanese Journal of Obstetrics & Gynecology, 2021
Objective: Polycystic ovary syndrome (PCOS) appears to be a common endocrine disorder of women in reproductive age. Adipose tissue (AT) is known as an active tissue in the metabolism of branched-chain amino acids (BCAA; Valine, Leucine, and Isoleucine ...
Shekoufeh Hajitarkhani, Ashraf Moini, Maryam Hafezi, Maryam Shahhoseini, AliReza Alizadeh   +4 more
doaj   +1 more source