Uroporphyrinogen-III synthase, ELISA Kit

RP11-124H7.1; OTTHUMP00000020710

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

This assay has high sensitivity and excellent specificity for detection of Mouse UROS. No significant cross-reactivity or interference between Mouse UROS and analogues was observed.

Store at 2-8 degree C.

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Small volumes of UROS elisa kit 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.

MBS284118 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Uroporphyrinogen-III synthase (UROS) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing UROS. The ELISA analytical biochemical technique of the MBS284118 kit is based on UROS antibody-UROS antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect UROS antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, UROS. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Principle of the Assay: This assay employs a two-site sandwich ELISA to quantitate UROS in samples. An antibody specific for UROS has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyUROS present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for UROS is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of UROS bound in the initial step. The color development is stopped and the intensity of the color is measured.

28,504 Da

uroporphyrinogen III synthase

uroporphyrinogen-III synthase

Uroporphyrinogen-III synthase

Uros3; UROIIIS; UROS  [Similar Products]

The protein encoded by this gene is the fourth enzyme in the heme biosynthesis pathway. It converts hydroxymethylbilane to uroporphyrinogen III, a cyclic tetrapyrrole. This enzyme is defective in the autosomal recessive disorder congenital erythropoietic porphyria. Alternate promoter usage controls cell type-specific expression, including erythroid cell-specific expression. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Sep 2014]

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; Lyase; Mitochondrial

Chromosomal Location of Human Ortholog: 7 F3|7 77.26 cM

Cellular Component: cytosol; mitochondrion

Molecular Function: cofactor binding; lyase activity; uroporphyrinogen-III synthase activity

Biological Process: heme biosynthetic process; porphyrin-containing compound biosynthetic process; tetrapyrrole biosynthetic process; uroporphyrinogen III biosynthetic process

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