Uroporphyrinogen III Synthase, Recombinant Protein

UROS Human; Uroporphyrinogen III Synthase Human Recombinant; Uroporphyrinogen-III synthase; UROIIIS; UROS; Hydroxymethylbilane hydrolyase [cyclizing]; Uroporphyrinogen-III cosynthase

For Research Use Only. Not for use in diagnostic procedures.

E Coli

Greater than 95.0% as determined by SDS-PAGE.

UROS protein solution (1mg/ml) containing 20mM Tris-HCl buffer (pH8.0), 10% glycerol and 0.1M NaCl.
Sterile filtered colorless solution.

UROS Human Recombinant although stable at 4 degree C for 1 week, should be stored below -18 degree C. Please prevent freeze thaw cycles.

Small volumes of UROS recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Description: UROS produced in E Coli is a single, non-glycosylated polypeptide chain containing 285 amino acids (1-265 a.a.) and having a molecular mass of 30.7kDa.UROS is fused to a 20 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Introduction: Uroporphyrinogen III synthase (UROS) is an enzyme involved in the 4th step of porphyrin metabolism and in the conversion of hydroxymethyl bilane into uroporphyrinogen III. Defects in the UROS protein can cause molecular lesions which lead to the autosomal recessive Gunther disease, otherwise known as congenital erythropoietic porphyria (CEP).

ENZYMES; Enzymes; Synthase

28,628 Da

uroporphyrinogen III synthase

uroporphyrinogen-III synthase; hydroxymethylbilane hydrolyase; uroporphyrinogen-III cosynthase

Uroporphyrinogen-III synthase

UROIIIS; UROS  [Similar Products]

HEM4_HUMAN

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]

UROS: Catalyzes cyclization of the linear tetrapyrrole, hydroxymethylbilane, to the macrocyclic uroporphyrinogen III, the branch point for the various sub-pathways leading to the wide diversity of porphyrins. Porphyrins act as cofactors for a multitude of enzymes that perform a variety of processes within the cell such as methionine synthesis (vitamin B12) or oxygen transport (heme). Defects in UROS are the cause of congenital erythropoietic porphyria (CEP); 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 ***** life. The deficiency in UROS activity results in the non-enzymatic conversion of hydroxymethylbilane (HMB) into the uroporphyrinogen-I isomer. Severe congenital erythropoietic porphyria is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non- immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders. Belongs to the uroporphyrinogen-III synthase family.

Protein type: Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; EC 4.2.1.75; Mitochondrial; Lyase

Chromosomal Location of Human Ortholog: 10q25.2-q26.3

Cellular Component: mitochondrion; cytosol

Molecular Function: uroporphyrinogen-III synthase activity; cofactor binding

Biological Process: response to antibiotic; uroporphyrinogen III biosynthetic process; porphyrin metabolic process; protoporphyrinogen IX biosynthetic process; heme biosynthetic process

Disease: Porphyria, Congenital Erythropoietic

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