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Mouse Anti-UROD Recombinant Antibody (2F5) (CBMAB-C4755-CN)

This product is a Mouse antibody that recognizes UROD. The antibody 2F5 can be used for immunoassay techniques such as: ELISA, WB.
See all UROD antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
2F5
Application
ELISA, WB

Basic Information

Immunogen
Recombinant protein corresponding to aa 268-367 of human UROD with GST tag (NP_000365)
Specificity
Human
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.4

Target

Full Name
uroporphyrinogen decarboxylase
Introduction
This gene encodes an enzyme in the heme biosynthetic pathway. This enzyme is responsible for catalyzing the conversion of uroporphyrinogen to coproporphyrinogen through the removal of four carboxymethyl side chains. Mutations and deficiency in this enzyme are known to cause familial porphyria cutanea tarda and hepatoerythropoetic porphyria.[provided by RefSeq, Aug 2010]
Entrez Gene ID
7389
UniProt ID
P06132
Alternative Names
Uroporphyrinogen Decarboxylase; EC 4.1.1.37; UPD; Uroporphyrinogen III Decarboxylase; URO-D; PCT;
Function
Catalyzes the sequential decarboxylation of the four acetate side chains of uroporphyrinogen to form coproporphyrinogen and participates in the fifth step in the heme biosynthetic pathway (PubMed:14633982, PubMed:11069625, PubMed:21668429, PubMed:11719352).
Isomer I or isomer III of uroporphyrinogen may serve as substrate, but only coproporphyrinogen III can ultimately be converted to heme (PubMed:14633982, PubMed:11069625, PubMed:21668429, PubMed:11719352).
In vitro also decarboxylates pentacarboxylate porphyrinogen I (PubMed:12071824).
Biological Process
Biological Process heme biosynthetic process Source:GO_Central1 Publication
Biological Process porphyrin-containing compound catabolic process Source:UniProtKB4 Publications
Biological Process porphyrin-containing compound metabolic process Source:UniProtKB1 Publication
Biological Process protoporphyrinogen IX biosynthetic process Source:UniProtKB-UniPathway
Cellular Location
Cytoplasm
Involvement in disease
Familial porphyria cutanea tarda (FPCT):
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. Familial porphyria cutanea tarda is an autosomal dominant disorder characterized by light-sensitive dermatitis, with onset in later life. It is associated with the excretion of large amounts of uroporphyrin in the urine. Iron overload is often present in association with varying degrees of liver damage.
Hepatoerythropoietic porphyria (HEP):
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. HEP is a cutaneous porphyria that presents in infancy. It is characterized biochemically by excessive excretion of acetate-substituted porphyrins and accumulation of protoporphyrin in erythrocytes. Uroporphyrinogen decarboxylase levels are very low in erythrocytes and cultured skin fibroblasts.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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