Acidosis and glucocorticoids (GC) are two catabolic signals associated with chronic renal disease. Previously, we reported that these signals stimulate branched-chain amino acid (BCAA) oxidation in renal tubule cells by increasing both the amount and activation state of branched-chain alpha-ketoacid dehydrogenase (BCKD). Activation of the BCKD complex could result from decreased expression of B...

Alternative Names MSUD Branched-Chain Ketoaciduria Branched-Chain Alpha-Keto Acid Dehydrogenase Deficiency BCKD Deficiency Keto Acid Decarboxylase Deficiency Maple Syrup Urine Disease, Classic Maple Syrup Urine Disease, Intermediate Maple Syrup Urine Disease, Intermittent Maple Syrup Urine Disease, Thiamine-Responsive Maple Syrup Urine Disease, E3-Deficient, with Lactic Acidosis Maple Syrup Uri...

Nellis, Mary M., Christopher B. Doering, Andrea Kasinski, and Dean J. Danner. Insulin increases branchedchain -ketoacid dehydrogenase kinase expression in Clone 9 rat cells. Am J Physiol Endocrinol Metab 283: E853–E860, 2002. First published June 3, 2002; 10.1152/ajpendo.00133.2002.— The branched-chain amino acids (BCAA) are committed to catabolism by the activity of the branched-chain -ketoaci...

The branched-chain amino acids (BCAA) accumulated in type 2 diabetes are independent contributors to insulin resistance. The activity of branched-chain α-keto acid dehydrogenase (BCKD) complex, rate-limiting enzyme in BCAA catabolism, is reduced in diabetic states, which contributes to elevated BCAA concentrations. However, the mechanisms underlying decreased BCKD activity remain poorly underst...

Branched chain amino acids (BCAAs) play critical roles in cell and tissue functions in addition to being important components of protein structure. The multifunctional roles speak to the need for maintaining tight control of their concentration within cells. As the BCAA cannot be made de novo in mammals, their cellular concentration is a function of dietary intake, endogenous protein turnover a...

Branched chain amino acids (BCAAs) play critical roles in cell and tissue functions in addition to being important components of protein structure. The multifunctional roles speak to the need for maintaining tight control of their concentration within cells. As the BCAA cannot be made de novo in mammals, their cellular concentration is a function of dietary intake, endogenous protein turnover a...

The branched-chain amino acids (BCAA) are essential amino acids required for protein homeostasis, energy balance, and nutrient signaling. In individuals with deficiencies in BCAA, these amino acids can be preserved through inhibition of the branched-chain-alpha-ketoacid dehydrogenase (BCKD) complex, the rate-limiting step in their metabolism. BCKD is inhibited by phosphorylation of its E1alpha ...

Wang, Xiaonan, and S. Russ Price. Differential regulation of branched-chain -ketoacid dehydrogenase kinase expression by glucocorticoids and acidification in LLC-PK1-GR101 cells. Am J Physiol Renal Physiol 286: F504–F508, 2004. First published November 11, 2003; 10.1152/ajprenal.00296.2003.—Acidosis and glucocorticoids (GC) are two catabolic signals associated with chronic renal disease. Previo...

Tandem mass spectrometry was applied to detect derangements in the pathways of amino acid and fatty acid metabolism in N-ethyl-N-nitrosourea-treated (ENU-treated) mice. We identified mice with marked elevation of blood branched-chain amino acids (BCAAs), ketoaciduria, and clinical features resembling human maple syrup urine disease (MSUD), a severe genetic metabolic disorder caused by the defic...

Leucine has been shown to stimulate adipose tissue protein synthesis in vivo as well as leptin secretion, protein synthesis, hyper-plastic growth, and tissue morphogenesis in in vitro experiments using freshly isolated adipocytes. Recently, others have proposed that leucine oxidation in the mitochondria may be required to activate the mammalian target of rapamycin (mTOR), the cytosolic Ser/Thr ...