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Mouse Anti-UROS Recombinant Antibody (CBCNC-819) (CBMAB-C4758-CN)

This product is a Mouse antibody that recognizes UROS. The antibody CBCNC-819 can be used for immunoassay techniques such as: WB.
See all UROS antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBCNC-819
Antibody Isotype
IgG1
Application
WB

Basic Information

Specificity
Human
Antibody Isotype
IgG1
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!]

Concentration
1 mg/mL

Target

Full Name
Uroporphyrinogen III Synthase
Introduction
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]
Entrez Gene ID
UniProt ID
Alternative Names
Uroporphyrinogen III Synthase; Uroporphyrinogen-III Cosynthase; UROIIIS; Hydroxymethylbilane Hydrolyase [Cyclizing]; Congenital Erythropoietic Porphyria; Hydroxymethylbilane Hydrolyase; Uroporphyrinogen-III Synthase; EC 4.2.1.75;
Function
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).
Biological Process
Biological Process cellular response to amine stimulus Source:Ensembl
Biological Process cellular response to arsenic-containing substance Source:Ensembl
Biological Process heme biosynthetic process Source:UniProtKB1 Publication
Biological Process protoporphyrinogen IX biosynthetic process Source:UniProtKB-UniPathway
Biological Process response to antibiotic Source:Ensembl
Biological Process response to platinum ion Source:Ensembl
Biological Process uroporphyrinogen III biosynthetic process Source:UniProtKB3 Publications
Cellular Location
cytosol
mitochondrion
Involvement in disease
Congenital erythropoietic porphyria (CEP):
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 adult life. The deficiency in UROS activity results in the non-enzymatic conversion of hydroxymethylbilane (HMB) into the uroporphyrinogen-I isomer.
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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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