|1.||Lee, Brendan: 4 articles (06/2013 - 10/2004)|
|2.||Erez, Ayelet: 2 articles (02/2011 - 09/2009)|
|3.||Brunetti-Pierri, Nicola: 2 articles (09/2009 - 10/2004)|
|4.||Jesudas, B: 1 article (11/2015)|
|5.||Raghu, R: 1 article (11/2015)|
|6.||Bhavani, G: 1 article (11/2015)|
|7.||Ezhilarasan, D: 1 article (11/2015)|
|8.||Karthikeyan, S: 1 article (11/2015)|
|9.||Russo, George: 1 article (10/2014)|
|10.||Legacy, Joseph: 1 article (10/2014)|
06/01/2013 - "Liver tests and argininosuccinic acid levels among patients with argininosuccinate lyase deficiency were unchanged or improved on GPB. "
02/15/2011 - "Deficiency of ASL results in an accumulation of argininosuccinic acid in tissues, and excretion of argininosuccinic acid in urine leading to the condition argininosuccinic aciduria (ASA). "
01/01/1985 - "Argininosuccinic aciduria, due to deficiency of argininosuccinic acid lyase, is generally associated with severe neonatal hyperammonaemia and its neurological sequelae. "
01/01/1972 - "New derivatives of argininosuccinic acid in the urine of a patient with argininosuccinicaciduria."
11/30/1993 - "An investigation of argininosuccinic acid anhydrides in argininosuccinic acid lyase deficiency by 1H-NMR spectroscopy."
01/01/2003 - "Metabolic studies of these mice demonstrated that they have the same biochemical phenotype as humans, with hyperammonemia, elevated plasma argininosuccinic acid and low plasma arginine. "
02/15/2011 - "While patients with ASA share the acute clinical phenotype of hyperammonemia, encephalopathy, and respiratory alkalosis common to other UCD, they also present with unique chronic complications most probably caused by a combination of tissue specific deficiency of arginine and/or elevation of argininosuccinic acid. "
09/01/2009 - "Argininosuccinic aciduria (ASA) is an inborn error of ureagenesis which if untreated leads to hyperammonemia, accumulation of argininosuccinic acid and arginine depletion. "
07/01/2007 - "Argininosuccinic aciduria (ASAuria) is an inborn error of metabolism caused by mutations in the argininosuccinate lyase (ASL) gene, which leads to the accumulation of argininosuccinic acid (ASA) in body fluids and severe hyperammonemia. "
08/01/2002 - "Hyperammonemia, elevation of glutamic acid and argininosuccinic acid and its anhydrides confirmed the diagnosis of ALD. "
01/01/1983 - "We monitored a pregnancy in a family at risk for citrullinemia due to argininosuccinic acid (ASA) synthetase deficiency. "
10/01/2014 - "Citrullinemia type I is an autosomal recessive disorder of the urea cycle in which a patient lacks the cytosolic enzyme, argininosuccinic acid synthetase. "
02/01/2002 - "Citrullinemia is a rare autosomal recessive inborn error of the urea cycle due to a deficiency in argininosuccinic acid synthetase. "
01/01/1996 - "Citrullinemia, a rare inborn error of metabolism, is characterized by a deficiency of argininosuccinic acid synthetase that results in large increases in plasma ammonia, citrulline, and glutamine, with normal acid-base balance. "
10/01/1995 - "The protocol involves repeated injections of INH over a 2-day period, resulting in significant hepatic necrosis as indicated by elevations of plasma argininosuccinic acid lyase activity. "
09/01/1997 - "In a rabbit model of isoniazid-induced hepatotoxicity, we have measured hepatic necrosis (quantitated by elevation of plasma argininosuccinic acid lyase (ASAL) activity), hepatic steatosis (quantitated by elevation of hepatic triglyceride content), and elevation in plasma triglyceride concentration in 15 rabbits. "
01/01/1996 - "Plasma hydrazine at 32 h was correlated significantly with plasma argininosuccinic acid lyase (ASAL, a sensitive marker of hepatic necrosis) activity as area under the curve (r2 = 0.54, P < 0.002) and log plasma ASAL activity at 48 h after the first dose of INH (r2 = 0.53, p < 0.005), but not with fatty changes. "
|5.||Inborn Urea Cycle Disorders
12/01/1990 - "Argininosuccinic acid lyase (ASAL) deficiency is a clinically heterogeneous autosomal recessive urea cycle disorder. "
01/01/1999 - "IEM identified were amino acid disorders eg phenylketonuria, maple syrup urine disease: urea cycle disorders eg ornithine transcarbamylase deficincy (OTC), argininosuccinic lyase deficiency (ALD), argininosuccinic acid synthetasc deficiency (ASD); glycogen storage disorders eg Pompe's discase, Von Gierkc's; organic acid disorders eg, isovaleric acidemia, methylmalonic acidemia. "
|5.||allatostatin 1 (ASAL)
|8.||Glutamic Acid (Glutamate)
|9.||Ornithine Carbamoyltransferase (Ornithine Transcarbamylase)