2-aminoadipic acid has been researched along with Zellweger Syndrome in 1 studies
2-Aminoadipic Acid: A metabolite in the principal biochemical pathway of lysine. It antagonizes neuroexcitatory activity modulated by the glutamate receptor, N-METHYL-D-ASPARTATE; (NMDA).
2-aminoadipic acid : An alpha-amino acid that is adipic acid bearing a single amino substituent at position 2. An intermediate in the formation of lysine.
Zellweger Syndrome: An autosomal recessive disorder due to defects in PEROXISOME biogenesis which involves more than 13 genes encoding peroxin proteins of the peroxisomal membrane and matrix. Zellweger syndrome is typically seen in the neonatal period with features such as dysmorphic skull; MUSCLE HYPOTONIA; SENSORINEURAL HEARING LOSS; visual compromise; SEIZURES; progressive degeneration of the KIDNEYS and the LIVER. Zellweger-like syndrome refers to phenotypes resembling the neonatal Zellweger syndrome but seen in children or adults with apparently intact peroxisome biogenesis.
| Excerpt | Relevance | Reference |
|---|---|---|
| "L-Pipecolic acid, a cyclic imino acid produced during the degradation of lysine, accumulates in body fluids of infants with the generalized peroxisomal disorders, including Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease." | 7.67 | Peroxisomal L-pipecolic acid oxidation is deficient in liver from Zellweger syndrome patients. ( Danks, DM; Mihalik, SJ; Moser, HW; Poulos, A; Rhead, WJ; Watkins, PA, 1989) |
| "L-Pipecolic acid, a cyclic imino acid produced during the degradation of lysine, accumulates in body fluids of infants with the generalized peroxisomal disorders, including Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease." | 3.67 | Peroxisomal L-pipecolic acid oxidation is deficient in liver from Zellweger syndrome patients. ( Danks, DM; Mihalik, SJ; Moser, HW; Poulos, A; Rhead, WJ; Watkins, PA, 1989) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 1 (100.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Mihalik, SJ | 1 |
| Moser, HW | 1 |
| Watkins, PA | 1 |
| Danks, DM | 1 |
| Poulos, A | 1 |
| Rhead, WJ | 1 |
1 other study available for 2-aminoadipic acid and Zellweger Syndrome
| Article | Year |
|---|---|
Peroxisomal L-pipecolic acid oxidation is deficient in liver from Zellweger syndrome patients.
Topics: 2-Aminoadipic Acid; Adult; Child; Child, Preschool; Humans; Infant; Liver; Microbodies; Oxidation-Re | 1989 |