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Literature summary for 3.6.4.6 extracted from

  • Imamura, A.; Tamura, S.; Shimozawa, N.; Suzuki, Y.; Zhang, Z.; Tsukamoto, T.; Orii, T.; Kondo, N.; Osumi, T.; Fujiki, Y.
    Temperature-sensitive mutation in PEX1 moderates the phenotypes of peroxisome deficiency disorders (1998), Hum. Mol. Genet., 7, 2089-2094.
    View publication on PubMed
Search for more literature for 3.6.4.6

Application

Application Comment Organism
medicine HsPEX1 is the causative gene for peroxisome-deficiency autosomal recessive disorders like cerebro-hepato-renal Zellweger syndrome, neonatal adrenoleukodystrophy and infantile refsum disease Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
cloning of PEX2, PEX6, PEX12 and PEX1 using yeast genes for homology search Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
ATP + H2O Homo sapiens
-
 ADP + phosphate
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Reaction

Reaction Comment Organism Reaction ID
ATP + H2O = ADP + phosphate large family of ATP-hydrolysing enzymes involved in the heterotypic fusion of membrane vesicles with target membranes and the homotypic fusion of various membrane compartments. They belong to the AAA-type (ATPase associated with a variety of cell activities) ATPase superfamily. They include peroxin, which apparently is involved in Zellweger 's syndrome. Homo sapiens
a

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast from PBD patients Homo sapiens
-
skin fibroblast
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + H2O
-
 Homo sapiens ADP + phosphate
-
 ?