Plasma Ammonia is Reduced by Glucose and Branched Chain Keto-Acids during Exercise in McArdle's Disease

1989 ◽  
Vol 76 (s20) ◽  
pp. 49P-49P ◽  
Author(s):  
JH Coakley ◽  
AJM Wagenmakers ◽  
RHT Edwards
Keyword(s):  
Plasma Ammonia ◽  
Mcardle's Disease ◽  
Keto Acids ◽  
Mcardle’S Disease ◽  
Branched Chain ◽  
Branched Chain Keto Acids

Relationship between ammonia, heart rate, and exertion in McArdle's disease

1992 ◽  
Vol 262 (2) ◽  
pp. E167-E172 ◽  
Author(s):  
J. H. Coakley ◽  
A. J. Wagenmakers ◽  
R. H. Edwards
Keyword(s):  
Heart Rate ◽  
Perceived Exertion ◽  
Total Work ◽  
Variable Effect ◽  
Plasma Ammonia ◽  
Mcardle's Disease ◽  
Mcardle’S Disease ◽  
Branched Chain ◽  
Deficiency Type ◽  
Type V

We studied plasma ammonia and exercise tolerance in six patients with McArdle's disease (myophosphorylase deficiency, type V glycogenosis) during incremental cycle ergometry. Tests were performed either in the postabsorptive state or after supplementation with branched-chain amino and 2-oxoacids and glucose. Glucose and branched-chain 2-oxoacid combined increased total work performed from control 49 +/- 22 to 80 +/- 36 kJ (P less than 0.05). Glucose alone also improved total work performed from 49 +/- 22 to 64 +/- 33 kJ (P less than 0.05). Branched-chain 2-oxoacids alone had a variable effect, and branched-chain amino acids were of no benefit. Correlations between plasma ammonia and heart rate for individual patients were r = 0.99, P less than 0.01; r = 0.95, P less than 0.01; r = 0.84, P less than 0.01; r = 0.76, P less than 0.01; r = 0.73, P less than 0.01; and r = 0.63, P less than 0.05 and between ammonia and perceived exertion for all patients combined was r = 0.70, P less than 0.0001. In two patients, correlation of ammonia with heart rate at a power output of 60 W was r = 0.91, P less than 0.001 and at 40 W was r = 0.77, P less than 0.001. We conclude that ammonia is either a mediator or a marker of the metabolic events leading to fatigue.

Influence of branched-chain amino acids and branched-chain keto acids on protein synthesis in isolated hepatocytes

Hepatology ◽  
1987 ◽  
Vol 7 (2) ◽  
pp. 324-329 ◽  
Author(s):  
Wolfgang Base ◽  
Carl Barsigian ◽  
Alisa Schaeffer ◽  
Ellen Shaw ◽  
Jose Martinez ◽  
...  
Keyword(s):  
Amino Acids ◽  
Protein Synthesis ◽  
Isolated Hepatocytes ◽  
Branched Chain Amino Acids ◽  
Keto Acids ◽  
Branched Chain ◽  
Branched Chain Keto Acids

Transamination of branched-chain keto acids by isolated perfused rat kidney.

1978 ◽  
Vol 235 (1) ◽  
pp. E47
Author(s):  
W E Mitch ◽  
W Chan
Keyword(s):  
Amino Acids ◽  
Rat Kidney ◽  
Branched Chain Amino Acids ◽  
Keto Acid ◽  
Branched Chain Amino Acid ◽  
Keto Acids ◽  
Perfused Rat Kidney ◽  
Branched Chain ◽  
Branched Chain Keto Acids ◽  
Isolated Rat

Isolated rat kidney perfused without substrate released serine, glycine, and taurine, and substantially smaller amounts of other amino acids. When branched-chain keto acids were added, the corresponding amino acids were released at rates amounting to 15-25% of keto acid disappearance. Perfusion with 2 mM alpha-keto-isovalerate or alpha-keto-beta-methylvalerate caused an increased glucose release amounting to 18-23% of keto acid disappearance. The activity of branched-chain amino acid transferase (BATase) was significantly stimulated by perfusion with the analogue of leucine, but not by perfusion with alpha-ketoglutarate, the analogues of valine or isoleucine, or with leucine itself. These findings document that the kidney converts branched-chain keto acids in part to the corresponding amino acids and suggest that the keto analogue of leucine may be involved in the control of renal BATase activity, thereby indirectly regulating the metabolism of branched-chain amino acids.

scholarly journals Branched-Chain Keto-Acids and Pyruvate in Blood: Measurement by HPLC with Fluorimetric Detection and Changes in Older Subjects

2000 ◽  
Vol 46 (6) ◽  
pp. 848-853 ◽  
Author(s):  
Karine Pailla ◽  
Françoise Blonde-Cynober ◽  
Christian Aussel ◽  
Jean-Pascal De Bandt ◽  
Luc Cynober
Keyword(s):  
Young Adults ◽  
Age Groups ◽  
Reversed Phase ◽  
Fluorometric Detection ◽  
Institutionalized Elderly ◽  
Keto Acids ◽  
Significant Difference ◽  
Branched Chain ◽  
Branched Chain Keto Acids

Abstract Background: Measurement of keto-acids is important in various clinical situations. The aim of the present work was to develop a rapid HPLC method for the determination of keto-acids in human serum and to assess the concentrations of these acids in young adults and institutionalized elderly adults. This method was applied to the determination of blood keto-acid concentrations of young adults and institutionalized elderly people, divided into age groups Methods: Four keto-acids (α-ketoisocaproate, α-ketoisovalerate, α-keto-β-methylvalerate, and pyruvate) were derivatized with o-phenylenediamine to give fluorescent derivatives. After the sample preparation step (75 min to prepare 20 samples), the derivatives were separated chromatographically on a reversed-phase column using a binary gradient. Results: The fluorometric detection of the four keto-acids was rapid, <12 min. The method is repeatable and reproducible: the CVs were <6% and <11%, respectively, for each of the keto-acids. We found no significant difference between males and females. Concentrations of the branched-chain keto-acids decreased after age 60 years, especially α-ketoisocaproate, which decreased ∼40%. Conclusions: The proposed method allows rapid and reliable measurement of keto-acids. The data demonstrate that changes in branched-chain keto-acids concentrations in serum occur with age.

New tools for an old question: dependence of ATP and bicarbonate for branched-chain keto acids oxidation

2019 ◽  
Vol 476 (15) ◽  
pp. 2235-2237
Author(s):  
Henver S. Brunetta ◽  
Graham P. Holloway
Keyword(s):  
Energy Production ◽  
Acid Oxidation ◽  
Methodological Issues ◽  
Amino Acid Oxidation ◽  
Branched Chain Amino Acid ◽  
Biochemical Journal ◽  
Keto Acids ◽  
Branched Chain ◽  
Branched Chain Keto Acids

Abstract Branched-chain keto acids (BCKA) metabolism involves several well-regulated steps within mitochondria, requires cofactors, and is modulated according to the metabolic status of the cells. This regulation has made it challenging to utilize in vitro approaches to determine the contribution of branched-chain amino acid oxidation to energy production. These methodological issues were elegantly addressed in a recent publication within the Biochemical Journal. In this issue, Goldberg et al. [Biochem. J. (2019) 476, 1521–1537] demonstrated in a well-designed system the dependence of ATP and bicarbonate for BCKA full oxidation. In addition, the utilized system allowed the authors to characterize specific biochemical routes within mitochondria for each BCKA. Among them, a quantitative analysis of the participation of BCKA on mitochondrial flux was estimated between tissues. These findings are milestones with meaningful impact in several fields of metabolism.

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