Reactome | Cytosolic uroporphyrinogen decarboxylase (UROD) catalyzes th...

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created	[InstanceEdit:189470] Jassal, B, 2006-11-20 11:41:56
dbId	189490
displayName	Cytosolic uroporphyrinogen decarboxylase (UROD) catalyzes th...
literatureReference	[LiteratureReference:190134] Purification and properties of uroporphyrinogen decarboxylase from human erythrocytes. A single enzyme catalyzing the four sequential decarboxylations of uroporphyrinogens I and III. [LiteratureReference:190137] Uroporphyrinogen decarboxylase: complete human gene sequence and molecular study of three families with hepatoerythropoietic porphyria [LiteratureReference:190161] Crystal structure of human uroporphyrinogen decarboxylase
modified	[InstanceEdit:2995335] Jassal, B, 2013-01-22
schemaClass	Summation
text	Cytosolic uroporphyrinogen decarboxylase (UROD) catalyzes the sequntial removal of four carboxylic groups from the acetic acid side chains of uroporphyrinogen III (URO3) to form coproporphyrinogen III (COPRO3) (de Verneuil et al. 1983). Human UROD is a dimer (Whitby et al. 1998). Heterogenous and homogenous deficiencies of UROD are associated with porphyria cutanea tarda and hepatoerythropoietic porphyria respectively in vivo (Moran-Jiminez et al. 1996).

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