KEGG   PATHWAY: map04146Help
Entry
map04146                    Pathway                                

Name
Peroxisome
Description
Peroxisomes are essential organelles that play a key role in redox signalling and lipid homeostasis. They contribute to many crucial metabolic processes such as fatty acid oxidation, biosynthesis of ether lipids and free radical detoxification. The biogenesis of peroxisomes starts with the early peroxins PEX3, PEX16 and PEX19 and proceeds via several steps. The import of membrane proteins into peroxisomes needs PEX19 for recognition, targeting and insertion via docking at PEX3. Matrix proteins in the cytosol are recognized by peroxisomal targeting signals (PTS) and transported to the docking complex at the peroxisomal membrane. Peroxisomes' deficiencies lead to severe and often fatal inherited peroxisomal disorders (PD). PDs are usually classified in two groups. The first group is disorders of peroxisome biogenesis which include Zellweger syndrome, and the second group is single peroxisomal enzyme deficiencies.
Class
Cellular Processes; Transport and catabolism
BRITE hierarchy
Pathway map
Peroxisome
map04146

All organismsOrtholog table
Disease
H00075  
Refsum disease
H00176  
Adrenoleukodystrophy (ALD)
H00177  
Adrenoleukodystrophy, neonatal (NALD)
H00203  
Acatalasemia
H00205  
Peroxisome biogenesis disorder
H00207  
Rhizomelic chondrodysplasia punctata
H00407  
Peroxisomal beta-oxidation enzyme deficiency
H00628  
Congenital bile acid synthesis defect (CBAS)
H00874  
Leukoencephalopathy with dystonia and motor neuropathy
H01155  
Roussy-Levy syndrome
H01283  
Malonyl-CoA decarboxylase deficiency
H01342  
Zellweger syndrome (ZS)
Other DBs
GO: 
Reference
  Authors
Wanders RJ, Waterham HR
  Title
Peroxisomal disorders: the single peroxisomal enzyme deficiencies.
  Journal
Biochim Biophys Acta 1763:1707-20 (2006)
DOI:10.1016/j.bbamcr.2006.08.010
Reference
  Authors
Steinberg SJ, Dodt G, Raymond GV, Braverman NE, Moser AB, Moser HW
  Title
Peroxisome biogenesis disorders.
  Journal
Biochim Biophys Acta 1763:1733-48 (2006)
DOI:10.1016/j.bbamcr.2006.09.010
Reference
  Authors
Rottensteiner H, Theodoulou FL
  Title
The ins and outs of peroxisomes: co-ordination of membrane transport and peroxisomal metabolism.
  Journal
Biochim Biophys Acta 1763:1527-40 (2006)
DOI:10.1016/j.bbamcr.2006.08.012
Reference
  Authors
Iida R, Yasuda T, Tsubota E, Takatsuka H, Matsuki T, Kishi K
  Title
Human Mpv17-like protein is localized in peroxisomes and regulates expression of antioxidant enzymes.
  Journal
Biochem Biophys Res Commun 344:948-54 (2006)
DOI:10.1016/j.bbrc.2006.04.008
Reference
  Authors
Kleinman R, Goulet OJ, Mieli-Vergani G, Sanderson I, Sherman P, Shneider B.
  Title
Walker's Pediatric Gastrointestinal Disease (5th edition)
  Journal
B.C.Decker (2008)
KO pathway
 

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