UROS Antibody

Code CSB-PA025679LA01HU
Size US$166
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) UROS Polyclonal antibody
Uniprot No.
Target Names
UROS
Alternative Names
congenital erythropoietic porphyria antibody; HEM4_HUMAN antibody; Hydroxymethylbilane hydrolyase [cyclizing] antibody; Hydroxymethylbilane hydrolyase antibody; OTTHUMP00000020709 antibody; OTTHUMP00000020710 antibody; UROIIIS antibody; Uroporphyrinogen III cosynthetase antibody; Uroporphyrinogen III synthase (congenital erythropoietic porphyria) antibody; Uroporphyrinogen III synthase antibody; Uroporphyrinogen-III cosynthase antibody; Uroporphyrinogen-III synthase antibody; UROS antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Uroporphyrinogen-III synthase protein (1-265AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The UROS Antibody (Product code: CSB-PA025679LA01HU) is Non-conjugated. For UROS Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA025679LB01HU UROS Antibody, HRP conjugated ELISA
FITC CSB-PA025679LC01HU UROS Antibody, FITC conjugated
Biotin CSB-PA025679LD01HU UROS Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form
Liquid
Tested Applications
ELISA
Protocols
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Customer Reviews and Q&A

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Target Background

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).
Gene References into Functions
  1. we show evidence that abnormal protein homeostasis is a prevalent mechanism responsible for UROS deficiency and that modulators of UROS proteolysis such as proteasome inhibitors or chemical chaperones may represent an attractive therapeutic option to reduce porphyrin accumulation and prevent skin photosensitivity in Congenital erythropoietic porphyria (CEP)patients when the genotype includes a missense variant. PMID: 28334762
  2. A, T to C change at nucleotide 34313, leading to a substitution of Leucine by Proline at codon 237, was observed in the homozygous state in a family with congenital erythropoietic porphyria. PMID: 22350154
  3. REVIEW: Structural, thermodynamic, and mechanistical studies in uroporphyrinogen III synthase and molecular basis of congenital erythropoietic porphyria PMID: 21570665
  4. Intracellular rescue of the uroporphyrinogen III synthase activity in enzymes carrying the hotspot mutation C73R. PMID: 21343304
  5. Data show that branchpoint sequence (BPS)mutation reduced the wild-type transcript and UROS enzyme activity in CEP lymphoblasts to approximately 10% and 15% of normal, respectively. PMID: 19965637
  6. two novel misense mutations in the UROS gene. PMID: 17298225
  7. NMR analyses of URO-synthase titrated with competitive inhibitors N(D)-methyl-1-formylbilane or URO'gen III, revealed resonance perturbations of specific residues lining the cleft between 2 major domains of URO synthase that mapped enzyme's active site. PMID: 18004775
  8. The decrease in the expression of ubiquitous HMBS and UROS mRNAs under hypoxia is associated with accumulation of hypoxia-inducible factor 1alpha protein. PMID: 19021769
  9. UROS mutations related to erythropoietic porphyria identify a key helix for protein stability. PMID: 19099412

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Involvement in disease
Congenital erythropoietic porphyria (CEP)
Protein Families
Uroporphyrinogen-III synthase family
Tissue Specificity
Ubiquitous.
Database Links

HGNC: 12592

OMIM: 263700

KEGG: hsa:7390

STRING: 9606.ENSP00000357775

UniGene: Hs.501376

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