UBR1 Antibody, FITC conjugated

說明書

中文名稱：

UBR1兔多克隆抗體, FITC偶聯
貨號：

CSB-PA812883LC01HU
規格：

￥880
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) UBR1 Polyclonal antibody
Uniprot No.：

Q8IWV7
基因名：

UBR1
別名：

E3 ubiquitin-protein ligase UBR1 antibody; JBS antibody; N-recognin-1 antibody; ubiquitin protein ligase E3 component n-recognin 1 antibody; Ubiquitin-protein ligase E3-alpha-1 antibody; Ubiquitin-protein ligase E3-alpha-I antibody; UBR1 antibody; UBR1_HUMAN antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human E3 ubiquitin-protein ligase UBR1 protein (722-862AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

FITC
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
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：

E3 ubiquitin-protein ligase which is a component of the N-end rule pathway. Recognizes and binds to proteins bearing specific N-terminal residues that are destabilizing according to the N-end rule, leading to their ubiquitination and subsequent degradation. May be involved in pancreatic homeostasis. Binds leucine and is a negative regulator of the leucine-mTOR signaling pathway, thereby controlling cell growth.
基因功能參考文獻：
1. UBR1 mutations of single or multi-exon deletions or duplications account for a substantial proportion of Johanson-Blizzard syndrome. PMID: 29178640
2. The frequency of any non-synonymous or synonymous variants was not different between the patients with chronic pancreatitis and controls PMID: 27397733
3. Reduced UBR1 expression affects MGMT turnover and DNA repair in the smokers lungs. PMID: 26183928
4. For all previously reported and novel UBR1 mutations together with their clinical data, a mutation database has been established at LOVD. PMID: 24599544
5. Testing the fetus and the affected sibling with recurrent Johanson-Blizzard syndrome revealed a homozygous truncating mutation in UBR1. PMID: 21711208
6. Results confirmed the relevance of specific missense UBR1 alleles to JBS, and suggested that a residual activity of a missense allele is causally associated with milder variants of JBS. PMID: 21931868
7. Molecular studies revealed a novel homozygous nonsense mutation in exon 38 of the UBR1 gene, which confirmed the diagnosis of Johanson-Blizzard syndrome. PMID: 20556423
8. Ubc2/Rad6 ser(120) regulates ubiquitin-dependent N-end rule targeting by E3{alpha}/Ubr1 PMID: 21041297
9. Deficiency of UBR1 perturbs the pancreas' acinar cells and other organs, presumably owing to metabolic stabilization of specific substrates of the N-end rule pathway. PMID: 16311597
10. Sustained ERK5 activity and the E3 ligase UBR1 regulate the stability and subcellular localization of c-Fos. PMID: 17018293
11. Thus we propose that autoISGylation of EFP negatively regulates its ISG15 E3 ligase activity for 14-3-3sigma. PMID: 17222803
12. These results suggest that Rabring7 is involved in the endocytic trafficking of EGFR through its E3 ligase activity. PMID: 17462600
13. Thiazolidinediones modulate the expression of beta catenin and other cell cycle proteins by targeting UBR1 independently of PPARG. PMID: 17569795
14. E3 ubiquitin ligase is an essential downstream component of the RAS signal transduction pathway. PMID: 18089810
15. study reports on two apparently unrelated girls with Johanson-Blizzard syndrome, in both children caused by a homozygous IVS26+5G>A mutation in the UBR1 gene PMID: 19006206
16. Case Report: Johanson-Blizzard syndrome with mild phenotypic features confirmed by UBR1 gene testing. PMID: 19058315
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相關疾病：

Johanson-Blizzard syndrome (JBS)
亞細胞定位：

Cytoplasm, cytosol.
蛋白家族：

UBR1 family
組織特異性：

Broadly expressed, with highest levels in skeletal muscle, kidney and pancreas. Present in acinar cells of the pancreas (at protein level).
數據庫鏈接：

HGNC: 16808

OMIM: 243800

KEGG: hsa:197131

STRING: 9606.ENSP00000290650

UniGene: Hs.591121

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