Immunogen
Synthesized peptide derived from human HEM4 AA range: 25-75
Specificity
This antibody detects endogenous levels of HEM4 at Human/Mouse
Source
Polyclonal, Rabbit,IgG
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB 1：500-2000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
Background
The protein encoded by this gene catalyzes the fourth step of porphyrin biosynthesis in the heme biosynthetic pathway. Defects in this gene cause congenital erythropoietic porphyria (Gunther's disease). [provided by RefSeq, Jul 2008],
Function
catalytic activity:Hydroxymethylbilane = uroporphyrinogen III + H(2)O.,disease:Defects in UROS are the cause of congenital erythropoietic porphyria (CEP) [MIM:263700]; also known as Gunther disease. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. The manifestations of CEP are heterogeneous, ranging from nonimmune hydrops fetalis due to severe hemolytic anemia in utero to milder, later onset forms, which have only skin lesions due to cutaneous photosensitivity in adult life. The deficiency in UROS activity results in the non-enzymatic conversion of hydroxymethylbilane (HMB) into the uroporphyrinogen-I isomer.,disease:Severe congenital erythropoietic
Fields
>>Porphyrin metabolism;>>Metabolic pathways;>>Biosynthesis of cofactors
Human protein sequence Database
P10746
Mouse protein sequence database
P51163
Rat protein sequence database
Cellular localization
mitochondrion,cytosol,
Tissue expression
Ubiquitous.
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