aspartic acid has been researched along with Maple Syrup Urine Disease in 8 studies
Aspartic Acid: One of the non-essential amino acids commonly occurring in the L-form. It is found in animals and plants, especially in sugar cane and sugar beets. It may be a neurotransmitter.
aspartic acid : An alpha-amino acid that consists of succinic acid bearing a single alpha-amino substituent
L-aspartic acid : The L-enantiomer of aspartic acid.
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)
| Excerpt | Relevance | Reference |
|---|---|---|
| "Cerebral cortex tissue was obtained at autopsy from neonatal Poll Hereford calves with clinically confirmed maple syrup urine disease (MSUD), neonatal Holstein-Friesian calves with clinically confirmed citrullinemia, and matched controls." | 7.68 | Glutamate and gamma-aminobutyric acid neurotransmitter systems in the acute phase of maple syrup urine disease and citrullinemia encephalopathies in newborn calves. ( Dennis, JA; Dodd, PR; Gundlach, AL; Harper, PA; Healy, PJ; Johnston, GA; Williams, SH, 1992) |
| "Cerebral cortex tissue was obtained at autopsy from neonatal Poll Hereford calves with clinically confirmed maple syrup urine disease (MSUD), neonatal Holstein-Friesian calves with clinically confirmed citrullinemia, and matched controls." | 3.68 | Glutamate and gamma-aminobutyric acid neurotransmitter systems in the acute phase of maple syrup urine disease and citrullinemia encephalopathies in newborn calves. ( Dennis, JA; Dodd, PR; Gundlach, AL; Harper, PA; Healy, PJ; Johnston, GA; Williams, SH, 1992) |
| "We describe a male neonate with classic maple syrup urine disease (MSUD) in metabolic crisis." | 1.40 | Neonatal case of classic maple syrup urine disease: usefulness of (1) H-MRS in early diagnosis. ( Adachi, M; Aida, N; Asakura, Y; Hanakawa, J; Hasegawa, T; Muroya, K; Sato, T; Tajima, G; Tomiyasu, M, 2014) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 2 (25.00) | 18.7374 |
| 1990's | 1 (12.50) | 18.2507 |
| 2000's | 1 (12.50) | 29.6817 |
| 2010's | 3 (37.50) | 24.3611 |
| 2020's | 1 (12.50) | 2.80 |
| Authors | Studies |
|---|---|
| Xu, J | 1 |
| Jakher, Y | 1 |
| Ahrens-Nicklas, RC | 1 |
| Muelly, ER | 1 |
| Moore, GJ | 1 |
| Bunce, SC | 1 |
| Mack, J | 1 |
| Bigler, DC | 1 |
| Morton, DH | 1 |
| Strauss, KA | 1 |
| Sato, T | 1 |
| Muroya, K | 1 |
| Hanakawa, J | 1 |
| Asakura, Y | 1 |
| Aida, N | 1 |
| Tomiyasu, M | 1 |
| Tajima, G | 1 |
| Hasegawa, T | 1 |
| Adachi, M | 1 |
| Terek, D | 1 |
| Koroglu, O | 1 |
| Yalaz, M | 1 |
| Gokben, S | 1 |
| Calli, C | 1 |
| Coker, M | 1 |
| Kultursay, N | 1 |
| Jan, W | 1 |
| Zimmerman, RA | 1 |
| Wang, ZJ | 1 |
| Berry, GT | 1 |
| Kaplan, PB | 1 |
| Kaye, EM | 1 |
| Prensky, AL | 1 |
| Moser, HW | 1 |
| Dodd, PR | 1 |
| Williams, SH | 1 |
| Gundlach, AL | 1 |
| Harper, PA | 1 |
| Healy, PJ | 1 |
| Dennis, JA | 1 |
| Johnston, GA | 1 |
| Levy, HL | 1 |
| Barkin, E | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Neuroimaging and Neuropsychological Outcomes in Urea Cycle Disorders[NCT02935283] | 56 participants (Anticipated) | Observational | 2016-08-31 | Recruiting | |||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
1 review available for aspartic acid and Maple Syrup Urine Disease
| Article | Year |
|---|---|
Brain Branched-Chain Amino Acids in Maple Syrup Urine Disease: Implications for Neurological Disorders.
Topics: Amino Acids, Branched-Chain; Aspartic Acid; Brain; Central Nervous System; Disease Susceptibility; G | 2020 |
7 other studies available for aspartic acid and Maple Syrup Urine Disease
| Article | Year |
|---|---|
Biochemical correlates of neuropsychiatric illness in maple syrup urine disease.
Topics: Adolescent; Adult; Affect; Anxiety; Aspartic Acid; Attention; Brain; Case-Control Studies; Child; Ch | 2013 |
Neonatal case of classic maple syrup urine disease: usefulness of (1) H-MRS in early diagnosis.
Topics: Aspartic Acid; Diagnosis, Differential; Early Diagnosis; Humans; Infant, Newborn; Lymphocytes; Male; | 2014 |
Diagnostic tools of early brain disturbances in an asymptomatic neonate with maple syrup urine disease.
Topics: Aspartic Acid; Brain Diseases; Brain Waves; Consanguinity; Developmental Disabilities; Diet; Diet, C | 2013 |
MR diffusion imaging and MR spectroscopy of maple syrup urine disease during acute metabolic decompensation.
Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Amino Acids, Branched-Chain; Aspartic Acid; Basal | 2003 |
Changes in the amino acid composition of proteolipids of white matter during maturation of the human nervous system.
Topics: Adult; Aged; Amino Acids; Aspartic Acid; Central Nervous System; Child; Frontal Lobe; Humans; Infant | 1967 |
Glutamate and gamma-aminobutyric acid neurotransmitter systems in the acute phase of maple syrup urine disease and citrullinemia encephalopathies in newborn calves.
Topics: Acute-Phase Reaction; Animals; Animals, Newborn; Aspartic Acid; Brain Diseases; Cattle; Cattle Disea | 1992 |
Comparison of amino acid concentrations between plasma and erythrocytes. Studies in normal human subjects and those with metabolic disorders.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids; Arginine; Aspartic Acid; Biological Transp | 1971 |