UROS Antibody

中文名稱：

UROS兔多克隆抗體
貨號：

CSB-PA025679LA01HU
規格：

￥440
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) UROS Polyclonal antibody
Uniprot No.：

P10746
基因名：

UROS
別名：

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
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Uroporphyrinogen-III synthase protein (1-265AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，UROS Antibody (CSB-PA025679LA01HU)，的標記方式是Non-conjugated。對于UROS Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產品名稱	應用
HRP	CSB-PA025679LB01HU	UROS Antibody, HRP conjugated	ELISA
FITC	CSB-PA025679LC01HU	UROS Antibody, FITC conjugated
Biotin	CSB-PA025679LD01HU	UROS Antibody, Biotin conjugated	ELISA

克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA
Protocols：

ELISA Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

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.
用途：

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

產品評價

靶點詳情

功能：

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).
基因功能參考文獻：
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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相關疾病：

Congenital erythropoietic porphyria (CEP)
蛋白家族：

Uroporphyrinogen-III synthase family
組織特異性：

Ubiquitous.
數據庫鏈接：

HGNC: 12592

OMIM: 263700

KEGG: hsa:7390

STRING: 9606.ENSP00000357775

UniGene: Hs.501376

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