pipecolic acid has been researched along with Cerebro-Hepato-Renal Syndrome in 8 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.
| 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) |
| "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) |
| "Progresses in biochemistry permit one to distinguish three biochemical forms of Zellweger Syndrome: 1) hyperpipecolic acidemia, 2) neonatal adrenoleukodystrophy, and 3) infantile Refsum's disease, which have similar clinical manifestations." | 3.67 | Zellweger syndrome, retinal involvement. ( Evrard, P; Stanesu-Segal, B, 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) |
| "PBD are divided into two types--Zellweger syndrome spectrum (ZSS) and rhizomelic chondrodysplasia punctata (RCDP)." | 2.43 | Peroxisome biogenesis disorders. ( Braverman, NE; Dodt, G; Moser, AB; Moser, HW; Raymond, GV; Steinberg, SJ, 2006) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 3 (37.50) | 18.7374 |
| 1990's | 4 (50.00) | 18.2507 |
| 2000's | 1 (12.50) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Steinberg, SJ | 1 |
| Dodt, G | 1 |
| Raymond, GV | 1 |
| Braverman, NE | 1 |
| Moser, AB | 1 |
| Moser, HW | 2 |
| Armstrong, DW | 1 |
| Zukowski, J | 1 |
| Ercal, N | 1 |
| Gasper, M | 1 |
| Baumgartner, MR | 1 |
| Verhoeven, NM | 1 |
| Jakobs, C | 1 |
| Roels, F | 1 |
| Espeel, M | 1 |
| Martinez, M | 1 |
| Rabier, D | 1 |
| Wanders, RJ | 1 |
| Saudubray, JM | 1 |
| Setchell, KD | 1 |
| Bragetti, P | 1 |
| Zimmer-Nechemias, L | 1 |
| Daugherty, C | 1 |
| Pelli, MA | 1 |
| Vaccaro, R | 1 |
| Gentili, G | 1 |
| Distrutti, E | 1 |
| Dozzini, G | 1 |
| Morelli, A | 1 |
| Broquist, HP | 1 |
| Roscher, AA | 1 |
| Hoefler, S | 1 |
| Hoefler, G | 1 |
| Paschke, E | 1 |
| Paltauf, F | 1 |
| Moser, A | 1 |
| Moser, H | 1 |
| Stanesu-Segal, B | 1 |
| Evrard, P | 1 |
| Mihalik, SJ | 1 |
| Watkins, PA | 1 |
| Danks, DM | 1 |
| Poulos, A | 1 |
| Rhead, WJ | 1 |
2 reviews available for pipecolic acid and Cerebro-Hepato-Renal Syndrome
| Article | Year |
|---|---|
Peroxisome biogenesis disorders.
Topics: Amino Acid Sequence; Chondrodysplasia Punctata, Rhizomelic; Humans; Membrane Proteins; Molecular Seq | 2006 |
Lysine-pipecolic acid metabolic relationships in microbes and mammals.
Topics: Animals; Fungi; Humans; Lysine; Mammals; Pipecolic Acids; Pseudomonas; Zellweger Syndrome | 1991 |
6 other studies available for pipecolic acid and Cerebro-Hepato-Renal Syndrome
| Article | Year |
|---|---|
Stereochemistry of pipecolic acid found in the urine and plasma of subjects with peroxisomal deficiencies.
Topics: Adult; Aged; Aged, 80 and over; Chromatography; Humans; Infant; Metabolism, Inborn Errors; Microbodi | 1993 |
Defective peroxisome biogenesis with a neuromuscular disorder resembling Werdnig-Hoffmann disease.
Topics: Bile Acids and Salts; Cells, Cultured; Diagnosis, Differential; Erythrocytes; Fatal Outcome; Fatty A | 1998 |
Oral bile acid treatment and the patient with Zellweger syndrome.
Topics: Administration, Oral; Bile Acids and Salts; Biopsy; Fatty Acids; Gas Chromatography-Mass Spectrometr | 1992 |
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 |
Zellweger syndrome, retinal involvement.
Topics: Bile Acids and Salts; Electroretinography; Fundus Oculi; Humans; Infant; Male; Pipecolic Acids; Reti | 1989 |
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 |