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Literature DB >> 3116331

Localization of pipecolic acid metabolism in rat liver peroxisomes: probable explanation for hyperpipecolataemia in Zellweger syndrome.

J M Trijbels¹, L A Monnens, G Melis, M van den Broekvan Essen, M Bruckwilder.

Abstract

The metabolism of [14C]pipecolic acid was studied in peroxisomal fractions of rat liver obtained by density gradient centrifugation in Percoll. The production rate of [14CO2] was used to measure the metabolic activity of the fractions towards [14C]carboxypipecolic acid as a substrate. It was shown that this activity was located in the peroxisomal fractions by comparison with the peroxisomal marker enzyme urate oxidase (EC 1.7.3.3). The process was markedly elevated by the addition of FAD. The apparent Km for DL-pipecolic acid was found to be 1.2 mmol L-1. Addition of ATP (1 mmol L-1) did not influence the decarboxylation rate of pipecolic acid. These results might explain the defective metabolism of pipecolic acid in patients with Zellweger syndrome who are lacking peroxisomes.

Entities: Chemical Disease Gene Species

Mesh：

Substances：

Year: 1987 PMID： 3116331 DOI： 10.1007/bf01800037

Source DB: PubMed Journal: J Inherit Metab Dis ISSN： 0141-8955 Impact factor: 4.982

14 in total

1. Cerebro-hepato-renal syndrome of Zellweger. A report of eight cases with comments upon the incidence, the liver lesion, and a fault in pipecolic acid metabolism.

Authors: D M Danks; P Tippett; C Adams; P Campbell
Journal: J Pediatr Date: 1975-03 Impact factor: 4.406

2. Infantile Refsum's disease: biochemical findings suggesting multiple peroxisomal dysfunction.

Authors: B T Poll-The; J M Saudubray; H Ogier; R B Schutgens; R J Wanders; G Schrakamp; H van den Bosch; J M Trijbels; A Poulos; H W Moser
Journal: J Inherit Metab Dis Date: 1986 Impact factor: 4.982

3. Conversion of 3 alpha, 7 alpha, 12 alpha-trihydroxy-5 beta-cholestanoic acid into cholic acid by rat liver peroxisomes.

Authors: J I Pedersen; J Gustafsson
Journal: FEBS Lett Date: 1980-12-01 Impact factor: 4.124

4. Evidence that peroxisomal acyl-CoA synthetase is located at the cytoplasmic side of the peroxisomal membrane.

Authors: G P Mannaerts; P Van Veldhoven; A Van Broekhoven; G Vandebroek; L J Debeer
Journal: Biochem J Date: 1982-04-15 Impact factor: 3.857

5. Cerebro-hepato-renal (Zellweger) syndrome and neonatal adrenoleukodystrophy: similarities in phenotype and accumulation of very long chain fatty acids.

Authors: F R Brown; A J McAdams; J W Cummins; R Konkol; I Singh; A B Moser; H W Moser
Journal: Johns Hopkins Med J Date: 1982-12

6. Pipecolic acid is oxidized by renal and hepatic peroxisomes. Implications for Zellweger's cerebro-hepato-renal syndrome (CHRS).

Authors: K Zaar; S Angermüller; A Völkl; H D Fahimi
Journal: Exp Cell Res Date: 1986-05 Impact factor: 3.905

Localization of pipecolic acid metabolism in rat liver peroxisomes: probable explanation for hyperpipecolataemia in Zellweger syndrome.

1. Cerebro-hepato-renal syndrome of Zellweger. A report of eight cases with comments upon the incidence, the liver lesion, and a fault in pipecolic acid metabolism.

2. Infantile Refsum's disease: biochemical findings suggesting multiple peroxisomal dysfunction.

3. Conversion of 3 alpha, 7 alpha, 12 alpha-trihydroxy-5 beta-cholestanoic acid into cholic acid by rat liver peroxisomes.

4. Evidence that peroxisomal acyl-CoA synthetase is located at the cytoplasmic side of the peroxisomal membrane.

5. Cerebro-hepato-renal (Zellweger) syndrome and neonatal adrenoleukodystrophy: similarities in phenotype and accumulation of very long chain fatty acids.

6. Pipecolic acid is oxidized by renal and hepatic peroxisomes. Implications for Zellweger's cerebro-hepato-renal syndrome (CHRS).

7. Biochemical studies in the liver and muscle of patients with Zellweger syndrome.

8. Serum very long chain fatty acid pattern in Zellweger syndrome.

9. Induction of peroxisomal beta-oxidation in rat liver by high-fat diets.

10. Hyperpipecolic acidemia in neonatal adrenoleukodystrophy.

1. Species variation in organellar location and activity of L-pipecolic acid oxidation in mammals.

Review 2. Reciprocal Control of Thyroid Binding and the Pipecolate Pathway in the Brain.