Uroporphyrinogen III Synthase (UROS), ELISA Kit

UROIIIS; Hydroxymethylbilane hydrolyase; Uroporphyrinogen-III cosynthase

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

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

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

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.

MBS456927 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 MBS456927 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(%).

Intended Uses: The kit is a sandwich enzyme immunoassay for the in vitro quantitative measurement of UROS in human serum, plasma, tissue homogenates and other biological fluids.

Principle of the Assay: The microtiter plate provided in this kit has been pre-coated with an antibody specific to UROS. Standards or samples are then added to the appropriate microtiter plate wells with a biotin-conjugated antibody preparation specific to UROS. Next, Avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. After TMB substrate solution is added, only those wells that contain UROS, biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. The enzyme-substrate reaction is terminated by the addition of sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450nm +/- 10nm. The concentration of UROS in the samples is then determined by comparing the O.D. of the samples to the standard curve.

28,628 Da

uroporphyrinogen III synthase

uroporphyrinogen-III synthase

Uroporphyrinogen-III synthase

UROIIIS; UROS  [Similar Products]

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; Lyase; Mitochondrial

Chromosomal Location of Human Ortholog: 10q26.2

Cellular Component: cytosol; mitochondrion

Molecular Function: cofactor binding; uroporphyrinogen-III synthase activity

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

Disease: Porphyria, Congenital Erythropoietic

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