glutaric acid has been researched along with Hepatolenticular Degeneration in 1 studies
glutaric acid: RN given refers to parent cpd
glutaric acid : An alpha,omega-dicarboxylic acid that is a linear five-carbon dicarboxylic acid.
Hepatolenticular Degeneration: A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC 3.6.3.4), also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as LIVER CIRRHOSIS; TREMOR; ATAXIA and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years.
| Excerpt | Relevance | Reference |
|---|---|---|
| "The cohort included patients with Leigh and Leigh-like syndrome (n = 25), other mitochondrial diseases (n = 25), Wilson disease (n = 40), Type 1 glutaric aciduria (n = 14), maple syrup urine disease (n = 13), giant axonal neuropathy (n = 5) and L-2 hydroxy glutaric aciduria (n = 3)." | 3.80 | Bilateral hypertrophic olivary nucleus degeneration on magnetic resonance imaging in children with Leigh and Leigh-like syndrome. ( Arvinda, HR; Bharath, MM; Bindu, PS; Gayathri, N; Govindaraju, C; Khan, NA; Nagappa, M; Ranjith, D; Sinha, S; Sonam, K; Taly, AB; Thangaraj, K, 2014) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 1 (100.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Bindu, PS | 1 |
| Taly, AB | 1 |
| Sonam, K | 1 |
| Govindaraju, C | 1 |
| Arvinda, HR | 1 |
| Gayathri, N | 1 |
| Bharath, MM | 1 |
| Ranjith, D | 1 |
| Nagappa, M | 1 |
| Sinha, S | 1 |
| Khan, NA | 1 |
| Thangaraj, K | 1 |
1 other study available for glutaric acid and Hepatolenticular Degeneration
| Article | Year |
|---|---|
Bilateral hypertrophic olivary nucleus degeneration on magnetic resonance imaging in children with Leigh and Leigh-like syndrome.
Topics: Child; Cohort Studies; Diagnosis, Differential; Early Diagnosis; Female; Giant Axonal Neuropathy; Gl | 2014 |