Porphobilinogen deaminase, ELISA Kit

Porphobilinogen deaminase; PBG-D; Hydroxymethylbilane synthase; HMBS; Pre-uroporphyrinogen synthase; HMBS; PBGD; UPS; 2.5.1.61

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

Store at 4 degree C

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Small volumes of HMBS 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.

MBS9427520 is a ready-to-use microwell, strip-or-full plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Porphobilinogen deaminase (HMBS) 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 HMBS. The ELISA analytical biochemical technique of the MBS9427520 kit is based on HMBS antibody-HMBS antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect HMBS antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, HMBS. 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(%).

33,264 Da

hydroxymethylbilane synthase

porphobilinogen deaminase

Porphobilinogen deaminase

PBGD; UPS; PBG-D; HMBS  [Similar Products]

This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008]

HMBS: Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps. Defects in HMBS are the cause of acute intermittent porphyria (AIP). AIP is a form of porphyria. 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. AIP is an autosomal dominant form of hepatic porphyria characterized by acute attacks of neurological dysfunctions with abdominal pain, hypertension, tachycardia, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. Belongs to the HMBS family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; EC 2.5.1.61; Mitochondrial; Transferase

Chromosomal Location of Human Ortholog: 11q23.3

Cellular Component: cytoplasm; cytosol

Molecular Function: hydroxymethylbilane synthase activity

Biological Process: heme biosynthetic process

Disease: Porphyria, Acute Intermittent

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