alanine has been researched along with Maple Syrup Urine Disease in 11 studies
Alanine: A non-essential amino acid that occurs in high levels in its free state in plasma. It is produced from pyruvate by transamination. It is involved in sugar and acid metabolism, increases IMMUNITY, and provides energy for muscle tissue, BRAIN, and the CENTRAL NERVOUS SYSTEM.
alanine : An alpha-amino acid that consists of propionic acid bearing an amino substituent at position 2.
Maple Syrup Urine Disease: An autosomal recessive inherited disorder with multiple forms of phenotypic expression, caused by a defect in the oxidative decarboxylation of branched-chain amino acids (AMINO ACIDS, BRANCHED-CHAIN). These metabolites accumulate in body fluids and render a maple syrup odor. The disease is divided into classic, intermediate, intermittent, and thiamine responsive subtypes. The classic form presents in the first week of life with ketoacidosis, hypoglycemia, emesis, neonatal seizures, and hypertonia. The intermediate and intermittent forms present in childhood or later with acute episodes of ataxia and vomiting. (From Adams et al., Principles of Neurology, 6th ed, p936)
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 8 (72.73) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 3 (27.27) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Bhattacharya, K | 1 |
| Khalili, V | 1 |
| Wiley, V | 1 |
| Carpenter, K | 1 |
| Wilcken, B | 1 |
| Wajner, A | 1 |
| Bürger, C | 1 |
| Dutra-Filho, CS | 1 |
| Wajner, M | 1 |
| de Souza Wyse, AT | 1 |
| Wannmacher, CM | 1 |
| Soltész, G | 1 |
| Jenkins, PA | 1 |
| Aynsley-Green, A | 1 |
| Morton, DH | 1 |
| Strauss, KA | 1 |
| Robinson, DL | 1 |
| Puffenberger, EG | 1 |
| Kelley, RI | 1 |
| Haymond, MW | 2 |
| Ben-Galim, E | 1 |
| Strobel, KE | 1 |
| Harper, PA | 1 |
| Dennis, JA | 1 |
| Healy, PJ | 1 |
| Brown, GK | 1 |
| François, J | 1 |
| Frimpter, GW | 1 |
| Karl, IE | 1 |
| Feigin, RD | 1 |
| DeVivo, D | 1 |
| Pagliara, AS | 1 |
| Boisse, J | 1 |
| Sietti, C | 1 |
3 reviews available for alanine and Maple Syrup Urine Disease
| Article | Year |
|---|---|
Ocular manifestations in aminoacidopathies.
Topics: Adult; Alanine; Albinism; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Cystinosis; | 1972 |
Aminoacidurias due to inherited disorders of metabolism. 2.
Topics: Acidosis; Alanine; Amino Acid Metabolism, Inborn Errors; Arginase; Arginine; Carbamates; Citrulline; | 1973 |
[New etiologic approach to periodic ketoacidosis in children].
Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Butyrates; Carbohydrate Metabolism, Inborn Errors; Ch | 1971 |
8 other studies available for alanine and Maple Syrup Urine Disease
| Article | Year |
|---|---|
Newborn screening may fail to identify intermediate forms of maple syrup urine disease.
Topics: Alanine; False Negative Reactions; Female; Humans; Infant, Newborn; Isoleucine; Leucine; Male; Maple | 2006 |
Synaptic plasma membrane Na(+), K (+)-ATPase activity is significantly reduced by the alpha-keto acids accumulating in maple syrup urine disease in rat cerebral cortex.
Topics: Alanine; Animals; Cell Membrane; Cerebral Cortex; Enzyme Activation; Hemiterpenes; Keto Acids; Maple | 2007 |
Hypoglycaemia in classical maple syrup urine disease is not due to hyperinsulinism.
Topics: Alanine; Female; Glucagon; Glucose Tolerance Test; Humans; Hypoglycemia; Infant; Insulin; Maple Syru | 1983 |
Diagnosis and treatment of maple syrup disease: a study of 36 patients.
Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Adult; Alanine; Amino Acids, Branched-Chain; Clin | 2002 |
Glucose and alanine metabolism in children with maple syrup urine disease.
Topics: Alanine; Child; Child, Preschool; Fasting; Female; Glucose; Humans; Kinetics; Male; Maple Syrup Urin | 1978 |
Maple syrup urine disease in calves: a clinical, pathological and biochemical study.
Topics: Alanine; Amino Acids, Branched-Chain; Animals; Breeding; Cattle; Cattle Diseases; Central Nervous Sy | 1989 |
Hypoglycemia and maple syrup urine disease: defective gluconeogenesis.
Topics: Alanine; Amino Acids; Chromatography; Diet Therapy; Female; Fructose; Glucagon; Gluconeogenesis; Glu | 1973 |
[Paper chromatography in the detection of aminoacidopathies].
Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Chromatography, Paper; Glycine; Histidin | 1971 |