Hyper-Beta-Alaninemia

02/01/1999 - "The present study indicates that BALA transaminase is involved in hyper-beta-alaninemia."
02/01/1999 - "Experimental beta-alaninuria was induced in rats by injection of (aminooxy)acetate (AOA), a potent inhibitor of aminotransferases, in order to elucidate the pathogenesis of hyper-beta-alaninemia. "
09/01/1994 - "We report intermittent seizures, lethargy, and Cohen's syndrome in a 4-year-old girl with hyper-beta-alaninemia and a partial deficiency of beta-alanyl-alpha-ketoglutarate transaminase (AKT). "

02/01/1999 - "The present study indicates that BALA transaminase is involved in hyper-beta-alaninemia."

08/01/1970 - "Abnormally high levels of lactate and pyruvate in cerebrospinal fluid of hyperalaninemia with hyperpyruvicemia."
11/01/1986 - "Laboratory work-up showed elevated blood lactate and pyruvate levels, a mild hyperalaninemia and hyperalaninuria and an increased urinary excretion of dicarboxylic acids. "
03/15/2004 - "The index patient had elevated liver enzymes as well as hyperalaninemia, lactic acidemia with an elevated lactate to pyruvate ratio, and increased urinary excretion of lactate, glutarate and Krebs cycle intermediates. "
05/04/1976 - "This infant was found to have lactic acidosis, low cerebrospinal fluid glucose, hyperalaninemia, and increased levels of urine lactate, pyruvate and alanine. "
06/01/2014 - "A 16month-old female child on antiepileptic drugs (valproate and topiramate) was suspected to have the inherited metabolic disorder, dihydrolipoamide dehydrogenase deficiency, based on clinical symptoms and metabolic profile showing hyperalaninemia, elevated branched-chain amino acids, and lactate-pyruvate ratio. "

08/01/1970 - "Abnormally high levels of lactate and pyruvate in cerebrospinal fluid of hyperalaninemia with hyperpyruvicemia."
11/01/1986 - "Laboratory work-up showed elevated blood lactate and pyruvate levels, a mild hyperalaninemia and hyperalaninuria and an increased urinary excretion of dicarboxylic acids. "
03/15/2004 - "The index patient had elevated liver enzymes as well as hyperalaninemia, lactic acidemia with an elevated lactate to pyruvate ratio, and increased urinary excretion of lactate, glutarate and Krebs cycle intermediates. "
05/04/1976 - "This infant was found to have lactic acidosis, low cerebrospinal fluid glucose, hyperalaninemia, and increased levels of urine lactate, pyruvate and alanine. "
06/01/2014 - "A 16month-old female child on antiepileptic drugs (valproate and topiramate) was suspected to have the inherited metabolic disorder, dihydrolipoamide dehydrogenase deficiency, based on clinical symptoms and metabolic profile showing hyperalaninemia, elevated branched-chain amino acids, and lactate-pyruvate ratio. "

05/01/2016 - "Hyper-beta-alaninemia is a rare metabolic condition that results in elevated plasma and urinary β-alanine levels and is characterized by neurotoxicity, hypotonia, and respiratory distress. "
05/01/2016 - "Mitochondrial defects associated with β-alanine toxicity: relevance to hyper-beta-alaninemia."
07/01/1991 - "Endotoxin treatment resulted in a state of hyperalaninemia and a threefold increase in hepatic alanine delivery (2.79 +/- 0.17 mu mole/100 g body weight/min in controls vs 8.13 +/- 0.98 in ENDO animals; P less than 0.001). "
10/01/1971 - "Glucagon could, therefore, be a humoral mediator of gluconeogenesis from endogenous alanine, responding to hyperalaninemia in the fasting state, but not when exogenous glucose is available."
05/04/1976 - "This infant was found to have lactic acidosis, low cerebrospinal fluid glucose, hyperalaninemia, and increased levels of urine lactate, pyruvate and alanine. "

05/01/1975 - "oral glucose load is retained within the splanchnic bed; (b) only 15 per cent of the ingested glucose is available for disposal by peripheral tissues as increased (above-basal) glucose utilization; (c) the height and shape or the oral glucose tolerance curve are largely determined by the rate and pattern of splanchnic glucose escape; (d) glucose-induced hyperlactatemia, hyperpyruvicemia and hyperalaninemia are due at least in part, to altered splanchnic exchange of these substrates."
10/01/1971 - "Glucagon could, therefore, be a humoral mediator of gluconeogenesis from endogenous alanine, responding to hyperalaninemia in the fasting state, but not when exogenous glucose is available."
05/04/1976 - "This infant was found to have lactic acidosis, low cerebrospinal fluid glucose, hyperalaninemia, and increased levels of urine lactate, pyruvate and alanine. "

11/01/1976 - "Hyperalaninemia and hyperlactatemia was higher in HH than in HCO groups and hyperglycemia was observed only in HH group (adrenergic stimulation). "
02/26/2013 - "Ten homozygous null (adenine nucleotide translocator-1(-/-)) patients monitored over a median of 6 years had a phenotype of progressive myocardial thickening, hyperalaninemia, lactic acidosis, exercise intolerance, and persistent adrenergic activation. "

05/01/1969 - "Pyruvic acidemia with hyperalaninemia: vitamin B1 dependency."
05/04/1976 - "Hyperalaninemia hyperpyruvicemia and lactic acidosis due to pyruvate carboxylase deficiency of the liver; treatment with thiamine and lipoic acid."

09/01/1994 - "Pyridoxine-responsive hyper-beta-alaninemia associated with Cohen's syndrome."
10/01/1983 - "The drug induced hyper beta-alaninemia was significantly (p less than or equal to 0.05) reduced in all three experiments by simultaneous pyridoxine administration in daily doses of 50 mg/kg B. "
10/01/1983 - "The effect of pyridoxine on 6-azauridine triacetate (6-AzUrd-TA) induced hyper beta-alaninemia was studied in New Zealand albino rabbits in three experiments. "
10/01/1983 - "6-azauridine triacetate induced hyper beta-alaninemia and its decrease by administration of pyridoxine."

10/01/1971 - "Glucagon could, therefore, be a humoral mediator of gluconeogenesis from endogenous alanine, responding to hyperalaninemia in the fasting state, but not when exogenous glucose is available."
11/01/1973 - "It is concluded that (a) glucocorticoids increase plasma glucagon concentration in the basal state and in response to protein ingestion or aminogenic stimulation; (b) this effect of glucocorticoids occurs in the face of obesity and persists in chronic hypercorticism; (c) hyperalaninemia is a characteristic of acute and chronic glucocorticoid excess, and may in turn contribute to steroid-induced hyperglucagonemia; and (d) increased alpha cell secretion may be a contributory factor in the gluconeogenic and diabetogenic effects of glucocorticoids."