|1.||Gospe, Sidney M: 3 articles (04/2011 - 04/2006)|
|2.||Kaczmarek, Piotr: 2 articles (10/2012 - 06/2011)|
|3.||Keay, Susan: 2 articles (10/2012 - 06/2011)|
|4.||Zhang, Chen-Ou: 2 articles (10/2012 - 06/2011)|
|5.||Plecko, B: 2 articles (06/2005 - 01/2005)|
|6.||Struys, E: 2 articles (06/2005 - 01/2005)|
|7.||Jakobs, C: 2 articles (06/2005 - 01/2005)|
|8.||Lanza, Elaine: 1 article (04/2015)|
|9.||Hartman, Terryl J: 1 article (04/2015)|
|10.||Murphy, Gwen: 1 article (04/2015)|
|1.||Zellweger Syndrome (Zellweger's Syndrome)
01/01/1980 - "Biochemical studies in the cerebro-hepato-renal syndrome of Zellweger: a disturbance in the metabolism of pipecolic acid."
10/01/1993 - "cerebralhepatorenyl (Zellweger) syndrome (CHRS) contained little D-pipecolic acid. "
05/01/1989 - "Thus, the high circulating levels of L-pipecolic acid in Zellweger syndrome probably result from defective peroxisomal oxidation of L-pipecolic acid to AAA."
05/01/1989 - "L-pipecolic acid oxidation in normal infant livers was low at birth and increased with age, but Zellweger syndrome livers showed little activity at any age. "
05/01/1989 - "Peroxisomal L-pipecolic acid oxidation is deficient in liver from Zellweger syndrome patients."
|2.||Peroxisomal Disorders (Peroxisomal Disorder)
01/01/2001 - "Biochemical studies revealed elevation of blood pipecolic acid and VLCFAs, compatible with peroxisomal disorder. "
02/01/2010 - "Determination of pipecolic acid following trimethylsilyl and trifluoroacyl derivatisation on plasma filter paper by stable isotope GC-MS for peroxisomal disorders."
07/01/2004 - "For all patients, pipecolic acid proved to be a useful parameter in the biochemical classification of peroxisomal disorders."
07/01/2004 - "In six patients the suggestion of a peroxisomal disorder was raised by the fortuitous finding of a pipecolic acid peak in amino acid chromatography, routinely performed as a general metabolic screening. "
07/01/2004 - "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. "
|3.||Liver Diseases (Liver Disease)
09/01/1999 - "Stereochemical studies of pipecolic acid were performed in patients with chronic liver diseases. "
03/01/1988 - "Plasma levels of pipecolic acid in patients with chronic liver disease."
09/01/1999 - "It is known that plasma pipecolic acid levels are elevated in patients with Zellweger syndrome, a genetic disorder, and chronic liver diseases. "
09/01/1999 - "Plasma levels of pipecolic acid, both L- and D-enantiomers, in patients with chronic liver diseases, especially hepatic encephalopathy."
03/01/1988 - "Plasma levels of pipecolic acid, which is a minor metabolite of lysine, were determined by high-performance liquid chromatography in 22 patients with chronic liver disease, composed of 6 patients with chronic active hepatitis, 11 with liver cirrhosis and 5 with hepatocellular carcinoma. "
06/01/2005 - "Determination of pipecolic acid in plasma and/or CSF should be included in the diagnostic work-up of patients with therapy-resistant seizures. "
08/16/1985 - "L-Pipecolic acid at the same dose given through the same route, however, shortened seizure latency significantly. "
04/01/2006 - "While the biochemical explanation for this finding is not clear, elevated pipecolic acid levels may serve as a diagnostic marker for patients with pyridoxine-dependent seizures. "
04/01/2006 - "Levels of both pipecolic acid and certain metabolites shown to be elevated in patients with PNPO mutations should be measured, and therapeutic trials of pyridoxal phosphate as well as pyridoxine should be considered early in the course of the management of infants and young children with intractable seizures."
01/01/1988 - "L-lysine, an essential amino acid for man and animals, and its metabolite pipecolic acid (PA) have been studied for their effects on pentylenetetrazol (PTZ)-induced seizures in mice. "
|5.||Refsum Disease (Refsum's Disease)
02/01/1988 - "Our data suggest that some patients with the infantile form of Refsum disease may show some clinical benefit from dietary management and this is reflected biochemically by decreases in the plasma levels of phytanic acid and pipecolic acid."
08/01/1986 - "In recent years a number of biochemical abnormalities have been described in patients with the infantile form of Refsum disease, including the accumulation of very long chain fatty acids, trihydroxycoprostanoic acid and pipecolic acid. "
12/01/1984 - "The plasma of patients with the infantile but not the adult form of Refsum's disease contains increased amounts of pipecolic acid and of at least two abnormal bile acids, one of which has been identified as 3 alpha, 7 alpha, 12 alpha trihydroxy-5 beta-cholestan-26-oic acid. "
10/01/1995 - "We present here genome wide linkage analysis of an atypical Refsum disease family where L-pipecolic acid level in blood was also increased, suggesting that the patients suffer from a new peroxisomal disorder intermediate between ARD and Infantile Refsum Disease (IRD, a peroxisomal deficiency disease). "
04/01/1988 - "In this study we carried out a detailed investigation into different peroxisomal functions in classical Refsum's disease by analyses of plasma (very long chain fatty acids, di- and trihydroxycoprostanoic acid and pipecolic acid) and cultured skin fibroblasts from the patients (de novo plasmalogen biosynthesis, very long chain fatty acid oxidation and amount of particle-bound catalase). "
|2.||Fatty Acids (Saturated Fatty Acids)
|5.||Uric Acid (Urate)
|6.||Serotonin (5 Hydroxytryptamine)
|7.||Pyridoxal Phosphate (Pyridoxal 5 Phosphate)