pipecolic acid has been researched along with Peroxisomal Disorders in 14 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.
Peroxisomal Disorders: A heterogeneous group of inherited metabolic disorders marked by absent or dysfunctional PEROXISOMES. Peroxisomal enzymatic abnormalities may be single or multiple. Biosynthetic peroxisomal pathways are compromised, including the ability to synthesize ether lipids and to oxidize long-chain fatty acid precursors. Diseases in this category include ZELLWEGER SYNDROME; INFANTILE REFSUM DISEASE; rhizomelic chondrodysplasia (CHONDRODYSPLASIA PUNCTATA, RHIZOMELIC); hyperpipecolic acidemia; neonatal adrenoleukodystrophy; and ADRENOLEUKODYSTROPHY (X-linked). Neurologic dysfunction is a prominent feature of most peroxisomal disorders.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Pipecolic acid was increased in all generalized peroxisomal disorders, while normal pipecolic acid with abnormal very long chain fatty acid concentrations was strong evidence for a single peroxisomal enzyme deficiency." | 5.32 | Hyperpipecolic acidaemia: a diagnostic tool for peroxisomal disorders. ( Baumgartner, MR; Bonetti, G; Jakobs, C; Nassogne, MC; Peduto, A; Poll-The, BT; Rabier, D; Saudubray, JM; Spada, M; Verhoeven, NM, 2004) |
| "Pipecolic acid (PA) is an important biochemical marker for the diagnosis of peroxisomal disorders." | 3.81 | Determination of plasma pipecolic acid by an easy and rapid liquid chromatography-tandem mass spectrometry method. ( Barraco, GM; Boenzi, S; Catesini, G; Dionisi-Vici, C; Iacovone, F; Inglese, R; Manco, M; Muraca, M; Rizzo, C; Semeraro, M, 2015) |
| "Pipecolic acid is a biochemical marker frequently detected in group 1 peroxisomal disorders (peroxisomal biogenesis disorders)." | 3.70 | Hyperpipecolic acidemia: clinical, biochemical, and radiologic observations. ( Al-Essa, MA; Chaves-Carballo, E; Ozand, PT, 1999) |
| "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) |
| "Pipecolic acid was increased in all generalized peroxisomal disorders, while normal pipecolic acid with abnormal very long chain fatty acid concentrations was strong evidence for a single peroxisomal enzyme deficiency." | 1.32 | Hyperpipecolic acidaemia: a diagnostic tool for peroxisomal disorders. ( Baumgartner, MR; Bonetti, G; Jakobs, C; Nassogne, MC; Peduto, A; Poll-The, BT; Rabier, D; Saudubray, JM; Spada, M; Verhoeven, NM, 2004) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 6 (42.86) | 18.2507 |
| 2000's | 5 (35.71) | 29.6817 |
| 2010's | 3 (21.43) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Semeraro, M | 1 |
| Muraca, M | 1 |
| Catesini, G | 1 |
| Inglese, R | 1 |
| Iacovone, F | 1 |
| Barraco, GM | 1 |
| Manco, M | 1 |
| Boenzi, S | 1 |
| Dionisi-Vici, C | 1 |
| Rizzo, C | 1 |
| Sorlin, A | 1 |
| Briand, G | 1 |
| Cheillan, D | 1 |
| Wiedemann, A | 1 |
| Montaut-Verient, B | 1 |
| Schmitt, E | 1 |
| Feillet, F | 1 |
| Steinberg, S | 1 |
| Jones, R | 1 |
| Tiffany, C | 1 |
| Moser, A | 1 |
| Yoon, HR | 1 |
| An, YW | 1 |
| Baas, JC | 1 |
| van de Laar, R | 1 |
| Dorland, L | 1 |
| Duran, M | 1 |
| Berger, R | 1 |
| Poll-The, BT | 2 |
| de Koning, TJ | 1 |
| Peduto, A | 1 |
| Baumgartner, MR | 1 |
| Verhoeven, NM | 2 |
| Rabier, D | 1 |
| Spada, M | 1 |
| Nassogne, MC | 1 |
| Bonetti, G | 1 |
| Jakobs, C | 3 |
| Saudubray, JM | 1 |
| Steinberg, SJ | 1 |
| Dodt, G | 2 |
| Raymond, GV | 1 |
| Braverman, NE | 1 |
| Moser, AB | 1 |
| Moser, HW | 1 |
| Vallat, C | 1 |
| Denis, S | 2 |
| Bellet, H | 1 |
| Wanders, RJ | 2 |
| Mion, H | 1 |
| Jansen, G | 1 |
| van Roermund, CW | 1 |
| Schutgens, RB | 1 |
| Jakobs, BS | 1 |
| Kulik, W | 1 |
| van den Heuvel, CM | 1 |
| Singh, I | 1 |
| Suzuki, Y | 1 |
| Al-Essa, MA | 1 |
| Chaves-Carballo, E | 1 |
| Ozand, PT | 1 |
| Kim, DG | 1 |
| Reimann, SA | 1 |
| Reuber, BE | 1 |
| McCabe, K | 1 |
| Gould, SJ | 1 |
| Mihalik, SJ | 1 |
3 reviews available for pipecolic acid and Peroxisomal Disorders
| Article | Year |
|---|---|
Peroxisome biogenesis disorders.
Topics: Amino Acid Sequence; Chondrodysplasia Punctata, Rhizomelic; Humans; Membrane Proteins; Molecular Seq | 2006 |
Biochemistry of peroxisomes in health and disease.
Topics: Animals; Arachidonic Acid; Bile Acids and Salts; Biological Transport; Cholesterol; Enzymes; Fatty A | 1997 |
[Other peroxisomal diseases].
Topics: Cholestanols; Humans; Oxidoreductases; Peroxisomal Disorders; Pipecolic Acids | 1998 |
11 other studies available for pipecolic acid and Peroxisomal Disorders
| Article | Year |
|---|---|
Determination of plasma pipecolic acid by an easy and rapid liquid chromatography-tandem mass spectrometry method.
Topics: Adolescent; Adult; Biomarkers; Calibration; Child; Child, Preschool; Chromatography, High Pressure L | 2015 |
Effect of l-Arginine in One Patient with Peroxisome Biogenesis Disorder due to PEX12 Deficiency.
Topics: Alanine Transaminase; Arginine; Aspartate Aminotransferases; Child; Child, Preschool; Deafness; Deve | 2016 |
Investigational methods for peroxisomal disorders.
Topics: Adrenoleukodystrophy; Fatty Acids; Gas Chromatography-Mass Spectrometry; Humans; Peroxisomal Disorde | 2008 |
Determination of pipecolic acid following trimethylsilyl and trifluoroacyl derivatisation on plasma filter paper by stable isotope GC-MS for peroxisomal disorders.
Topics: Acetamides; Adolescent; Adult; Child; Child, Preschool; Clinical Laboratory Techniques; Fluoroacetat | 2010 |
Plasma pipecolic acid is frequently elevated in non-peroxisomal disease.
Topics: Biomarkers; False Positive Reactions; Humans; Metabolism, Inborn Errors; Peroxisomal Disorders; Pipe | 2002 |
Hyperpipecolic acidaemia: a diagnostic tool for peroxisomal disorders.
Topics: Adolescent; Adult; Bile Acids and Salts; Chromatography; Fatty Acids; Female; France; Gas Chromatogr | 2004 |
Major hyperpipecolataemia in a normal adult.
Topics: Adult; Humans; Lysine; Male; Peroxisomal Disorders; Pipecolic Acids; Stereoisomerism | 1996 |
Metabolic aspects of peroxisomal disorders.
Topics: Cholesterol; Fatty Acids; Fatty Acids, Unsaturated; Humans; Peroxisomal Disorders; Phospholipids; Ph | 1996 |
Pre- and postnatal diagnosis of peroxisomal disorders using stable-isotope dilution gas chromatography--mass spectrometry.
Topics: Amniotic Fluid; Bile Acids and Salts; Fatty Acids; Female; Gas Chromatography-Mass Spectrometry; Hum | 1995 |
Hyperpipecolic acidemia: clinical, biochemical, and radiologic observations.
Topics: Brain; Child, Preschool; Humans; Infant, Newborn; Male; Peroxisomal Disorders; Pipecolic Acids; Tomo | 1999 |
L-Pipecolic acid oxidase, a human enzyme essential for the degradation of L-pipecolic acid, is most similar to the monomeric sarcosine oxidases.
Topics: Amino Acid Sequence; Animals; Carrier Proteins; Cloning, Molecular; Haplorhini; Humans; Kidney; Live | 2000 |