Recombinant Human Atrophin-1 (ATN1), partial

中文名稱：

Recombinant Human Atrophin-1(ATN1) ,partial
貨號：

CSB-YP002305HU
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

Recombinant Human Atrophin-1(ATN1) ,partial
貨號：

CSB-EP002305HU
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

Recombinant Human Atrophin-1(ATN1) ,partial
貨號：

CSB-EP002305HU-B
說明書：

MSDS Datasheet
規格：
來源：

E.coli
共軛：

Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
其他：

中文名稱：

Recombinant Human Atrophin-1(ATN1) ,partial
貨號：

CSB-BP002305HU
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

Recombinant Human Atrophin-1(ATN1) ,partial
貨號：

CSB-MP002305HU
說明書：

MSDS Datasheet
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

ATN1
Uniprot No.：

P54259
別名：

ATN1; D12S755E; DRPLAAtrophin-1; Dentatorubral-pallidoluysian atrophy protein
種屬：

Homo sapiens (Human)
蛋白長度：

Partial
蛋白標簽：

Tag?type?will?be?determined?during?the?manufacturing?process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
產品提供形式：

Lyophilized powder Warning: in_array() expects parameter 2 to be array, null given in /www/web/cusabio_cn/public_html/caches/caches_template/default/content/show_product_protein.php on line 662
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
復溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
儲存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet ：

Please contact us to get it.

產品評價

靶點詳情

功能：

Transcriptional corepressor. Recruits NR2E1 to repress transcription. Promotes vascular smooth cell (VSMC) migration and orientation. Corepressor of MTG8 transcriptional repression. Has some intrinsic repression activity which is independent of the number of poly-Gln (polyQ) repeats.
基因功能參考文獻：
1. Data indicate that the size of the expanded CAG repeats od dentatorubral-pallidoluysian atrophy protein (DRPLA) was inversely correlated with the age at onset. PMID: 27577205
2. Epigenetic regulation of ATN1 by LSD1 is required for cortical progenitor maintenance. PMID: 25519973
3. Juvenile myoclonic epilepsy is not associated with the DRPLA gene in a European population. PMID: 25398822
4. These data demonstrate that the expanded trinucleotide repeat within ATN-1 mRNA is a potential target for compounds designed to achieve allele-selective inhibition of ATN-1 protein, and one agent may allow the targeting of multiple disease genes. PMID: 24981774
5. results suggest that expanded polyQ repeats in ATN1 may contribute to neurodegeneration via alterations in both protein aggregation and intracellular localization. PMID: 23933208
6. This study demonistrated that hypoalbuminemia in early onset DRPLA revealed the possibility of multiorgan involvement. PMID: 23263592
7. In cerebrum and cerebellum of DRPLA transgenic mouse lines at 4, 8, and 12 weeks it is demonstrated that both the number and expression levels of the altered genes are highly dependent on CAG repeat length and age in both brain regions. PMID: 22342974
8. This study demonistrated that Atn1 is response for Dentatorubral-pallidoluysian atrophy. PMID: 22083836
9. A pair of monozygotic twins is diagnosed with dentatorubro pallidoluysian atrophy, identical CAG repeats, and different clinical courses. PMID: 21749564
10. This sttudy suggested that age-dependent and CAG repeat-dependent intranuclear accumulation of mutant DRPLA leading to nuclear dysfunctions are suggested to be the essential pathophysiologic mechanisms in Dentatorubral-pallidoluysian atrophy. PMID: 21827919
11. Atrophin-1 promote neurodegeneration with autophagic hallmarks both in neuronal photoreceptors and glial cells. PMID: 20339376
12. the C-terminal fragment plays a principal role in the pathological accumulation of ATN1 in dentatorubral-pallidoluysian atrophy PMID: 20977674
13. The CAG repeat length of DRPLA may have a considerable effect on not only the disease onset but also the disease milestones and prognosis in DRPLA patients. PMID: 20589872
14. Molecular testing for dentatorubral pallidoluysian atrophy revealed abnormal "cytosine-adenine-guanosine" expansion in the atrophin-1 gene. PMID: 20196398
15. Nuclear localization of a non-caspase truncation product of this protein, with an expanded polyglutamine repeat, increases cellular toxicity. PMID: 12464607
16. DRPLA protein is a phosphoprotein, and c-Jun NH(2)-terminal kinase (JNK) is one of the major factors involved in its phosphorylation PMID: 12812981
17. The molecular architecture of CAG repeats in mutant DRPLA was studied. PMID: 15223312
18. In DRPLA, the expression ratio of two mRNA isoforms was generated by alternative splicing. The glutamine-included protein isoform was more predominantly localized in the cell nucleus. PMID: 16091834
19. mutant atropphin with polyglutamine expansion does not simply function in a dominant negative manner PMID: 16407196
20. Status epilepticus during sleep caused by an expansion of the CAG repeat in the DRPLA gene. PMID: 16534126
21. linkage and association for three CAG triplet repeat markers (Spinocerebellar Ataxia Type 1, SCA1; Machado-Joseph Disease, MJD; Dentatorubro-pallidoluysian Atrophy, DRPLA) to assess their contribution to variation in cognitive ability PMID: 16967484
22. Both Drosophila and human Brakeless and Atrophin interact in vitro. Brakeless homologs may influence the toxicity of polyglutamine-expanded Atrophin-1. PMID: 17503969
23. data suggest that a founder effect accounts for some of the high prevalence of Dentatorubral pallidoluysian and ATN1 expansion polymorphism in Wales. PMID: 17965145
24. 244 Patients with the diagnosis of Huntington's Disease and without mutation of the IT15 gene revealed one case of SCA17 but did not disclose the presence of two other diseases with a similar clinical manifestation: DRPLA and HDL2. PMID: 18651325
25. The dentatorubral-pallidoluysian atrophy transgenic mice showed severe neurological phenotypes with progressive brain atrophy and premature death. PMID: 19039037
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相關疾病：

Dentatorubral-pallidoluysian atrophy (DRPLA)
亞細胞定位：

Nucleus. Cytoplasm, perinuclear region. Cell junction.
組織特異性：

Widely expressed in various tissues including heart, lung, kidney, ovary, testis, prostate, placenta, skeletal Low levels in the liver, thymus and leukocytes. In the adult brain, broadly expressed in amygdala, caudate nucleus, corpus callosum, hippocampus
數據庫鏈接：

HGNC: 3033

OMIM: 125370

KEGG: hsa:1822

STRING: 9606.ENSP00000349076

UniGene: Hs.143766