pipecolic acid has been researched along with Adrenoleukodystrophy in 7 studies
pipecolic acid: RN given refers to cpd without isomeric designation
pipecolic acid : A piperidinemonocarboxylic acid in which the carboxy group is located at position C-2.
pipecolate : A piperidinecarboxylate that is the conjugate base of pipecolic acid.
Adrenoleukodystrophy: An X-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the LYSOSOMES of ADRENAL CORTEX and the white matter of CENTRAL NERVOUS SYSTEM. This disease occurs almost exclusively in the males. Clinical features include the childhood onset of ATAXIA; NEUROBEHAVIORAL MANIFESTATIONS; HYPERPIGMENTATION; ADRENAL INSUFFICIENCY; SEIZURES; MUSCLE SPASTICITY; and DEMENTIA. The slowly progressive adult form is called adrenomyeloneuropathy. The defective gene ABCD1 is located at Xq28, and encodes the adrenoleukodystrophy protein (ATP-BINDING CASSETTE TRANSPORTERS).
| Excerpt | Relevance | Reference |
|---|---|---|
| "Serum pipecolic acid was measured in patients with neonatal adrenoleukodystrophy (NALD), sex-linked ALD, and the cerebrohepatorenal syndrome of Zellweger." | 7.67 | Hyperpipecolic acidemia in neonatal adrenoleukodystrophy. ( Kelley, RI; Moser, HW, 1984) |
| "Cerebro-hepato-renal (Zellweger) syndrome, adrenoleukodystrophy, and Refsum's disease patients can be divided into at least five distinct groups, according to the nature of their plasma changes and their fibroblast phytanic acid oxidase activities." | 7.67 | Cerebro-hepato-renal (Zellweger) syndrome, adrenoleukodystrophy, and Refsum's disease: plasma changes and skin fibroblast phytanic acid oxidase. ( Danks, DM; Fellenberg, AJ; Poulos, A; Sharp, P, 1985) |
| "Serum pipecolic acid was measured in patients with neonatal adrenoleukodystrophy (NALD), sex-linked ALD, and the cerebrohepatorenal syndrome of Zellweger." | 3.67 | Hyperpipecolic acidemia in neonatal adrenoleukodystrophy. ( Kelley, RI; Moser, HW, 1984) |
| "Cerebro-hepato-renal (Zellweger) syndrome, adrenoleukodystrophy, and Refsum's disease patients can be divided into at least five distinct groups, according to the nature of their plasma changes and their fibroblast phytanic acid oxidase activities." | 3.67 | Cerebro-hepato-renal (Zellweger) syndrome, adrenoleukodystrophy, and Refsum's disease: plasma changes and skin fibroblast phytanic acid oxidase. ( Danks, DM; Fellenberg, AJ; Poulos, A; Sharp, P, 1985) |
| "Disorders of peroxisomal biogenesis include the Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum syndrome, and hyperpipecolic acidemia." | 3.67 | Genetic and phenotypic heterogeneity in disorders of peroxisome biogenesis--a complementation study involving cell lines from 19 patients. ( Hoefler, G; Hoefler, S; Moser, A; Moser, H; Paltauf, F; Paschke, E; Roscher, AA, 1989) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 6 (85.71) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 1 (14.29) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Steinberg, S | 1 |
| Jones, R | 1 |
| Tiffany, C | 1 |
| Moser, A | 2 |
| Kelley, RI | 1 |
| Moser, HW | 3 |
| Poulos, A | 1 |
| Sharp, P | 1 |
| Fellenberg, AJ | 1 |
| Danks, DM | 1 |
| Monnens, L | 1 |
| Heymans, H | 1 |
| Roscher, AA | 1 |
| Hoefler, S | 1 |
| Hoefler, G | 1 |
| Paschke, E | 1 |
| Paltauf, F | 1 |
| Moser, H | 1 |
| Goldfischer, S | 1 |
| Collins, J | 1 |
| Rapin, I | 1 |
| Coltoff-Schiller, B | 1 |
| Chang, CH | 1 |
| Nigro, M | 1 |
| Black, VH | 1 |
| Javitt, NB | 1 |
| Lazarow, PB | 1 |
1 review available for pipecolic acid and Adrenoleukodystrophy
| Article | Year |
|---|---|
Peroxisomal disorders: clinical characterization.
Topics: Adrenoleukodystrophy; Brain Diseases; Chondrodysplasia Punctata; Enzymes; Humans; Hyperoxaluria, Pri | 1987 |
6 other studies available for pipecolic acid and Adrenoleukodystrophy
| Article | Year |
|---|---|
Investigational methods for peroxisomal disorders.
Topics: Adrenoleukodystrophy; Fatty Acids; Gas Chromatography-Mass Spectrometry; Humans; Peroxisomal Disorde | 2008 |
Hyperpipecolic acidemia in neonatal adrenoleukodystrophy.
Topics: Abnormalities, Multiple; Adrenoleukodystrophy; Brain; Child; Child, Preschool; Diffuse Cerebral Scle | 1984 |
Cerebro-hepato-renal (Zellweger) syndrome, adrenoleukodystrophy, and Refsum's disease: plasma changes and skin fibroblast phytanic acid oxidase.
Topics: Adrenoleukodystrophy; Brain Diseases; Cells, Cultured; Child, Preschool; Chromatography, Gas; Diffus | 1985 |
Peroxisomal disorders.
Topics: Adrenoleukodystrophy; Animals; Brain Diseases; Child; Child, Preschool; Chondrodysplasia Punctata; H | 1986 |
Genetic and phenotypic heterogeneity in disorders of peroxisome biogenesis--a complementation study involving cell lines from 19 patients.
Topics: Adrenoleukodystrophy; Cell Line; Child; Child, Preschool; Diffuse Cerebral Sclerosis of Schilder; Fe | 1989 |
Peroxisomal defects in neonatal-onset and X-linked adrenoleukodystrophies.
Topics: Adrenoleukodystrophy; Adult; Animals; Bile Acids and Salts; Catalase; Child; Child, Preschool; Diffu | 1985 |