磷酸化脊髓小腦失調(diào)癥蛋白1抗體 返回列表頁(yè)

英文名稱 phospho-Ataxin 1(Ser775)
中文名稱 磷酸化脊髓小腦失調(diào)癥蛋白1抗體
別    名 Ataxin 1 (phospho S776);p-Ataxin 1 (phospho S776); ATX1; ATXN1; SCA1; Ataxin 1; Ataxin-1; Ataxin1; Spinocerebellar ataxia type 1; ATX1_HUMAN.
磷酸化脊髓小腦失調(diào)癥蛋白1抗體  
說(shuō) 明 書 0.1ml  
產(chǎn)品類型 磷酸化抗體 
研究領(lǐng)域 細(xì)胞生物  神經(jīng)生物學(xué)  表觀遺傳學(xué)  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, 
產(chǎn)品應(yīng)用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復(fù)）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 87kDa
細(xì)胞定位 細(xì)胞核 細(xì)胞漿 
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthesised phosphopeptide derived from human Ataxin 1 around the phosphorylation site of Ser776
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 background:
Ataxin-1, also designated spinocerebellar ataxia type 1 protein (Sca-1), is differentially expressed and localizes to both the cytoplasm and the nucleus. Mutations in Ataxin-1 are associated with the onset of the autosomal dominant neurodegenerative disorder spinocerebellar ataxia type 1 (SCA-1), which is characterized by progressive neuronal loss in the cerebellum, muscle wasting and ataxia. In Purkinje cells, where SCA-1 is predominantly observed, Ataxin-1 has been shown to directly associate with the Purkinje-enriched leucine-rich acidic nuclear protein (LANP) and the nuclear matrix-associated protein promyelocytic leukemia protein PML. In SCA-1, Ataxin-1 is mutated to encode a polyglutamine protein that forms nuclear aggregates, which interact significantly more strongly with LANP and contribute to the pathogenesis of SCA-1.

Function:
Binds RNA in vitro. May be involved in RNA metabolism. The expansion of the polyglutamine tract may alter this function.

Subunit:
Homooligomer. Interacts with CIC (By similarity). Interacts with ANP32A, PQBP1, UBQLN4, ATXN1L, USP7 and ZNF804A. Directly interacts with RBPJ; this interaction is disrupted in the presence of Notch intracellular domain. Competes with ATXN1L for RBPJ-binding.

Subcellular Location:
Cytoplasm. Nucleus. Colocalizes with USP7 in the nucleus.

Tissue Specificity:
Widely expressed throughout the body.

Post-translational modifications:
Phosphorylation at Ser-775 increases the pathogenicity of proteins with an expanded polyglutamine tract.
Sumoylation is dependent on nuclear localization and phosphorylation at Ser-775. It is reduced in the presence of an expanded polyglutamine tract.

DISEASE:
Defects in ATXN1 are the cause of spinocerebellar ataxia type 1 (SCA1) [MIM:164400]; also known as olivopontocerebellar atrophy I (OPCA I or OPCA1). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to cerebellum degeneration with variable involvement of the brainstem and spinal cord. SCA1 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. SCA1 is caused by expansion of a CAG repeat in the coding region of ATXN1. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.

Similarity:
Belongs to the ATXN1 family.
Contains 1 AXH domain.

Gene ID:
6310

Database links:

Entrez Gene: 6310 Human

Entrez Gene: 20238 Mouse

Entrez Gene: 25049 Rat

Omim: 601556 Human

SwissProt: P54253 Human

SwissProt: P54254 Mouse

SwissProt: Q63540 Rat

Unigene: 434961 Human

Unigene: 342683 Mouse

Unigene: 342686 Mouse

Unigene: 88438 Rat

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.xy-11138R PCDHB4原鈣粘蛋白β4抗體
xy-11143R PCDHGA10原鈣粘蛋白γ10抗體
xy-11144R PCDHGA4原鈣粘蛋白γA4抗體
xy-11145R PCDHGA2原鈣粘蛋白γA2抗體
xy-11147R PCDHGA5原鈣粘蛋白γA5抗體
xy-11148R PCDHGA7原鈣粘蛋白γA7抗體
xy-11149R PCDHGA8原鈣粘蛋白γA8抗體
xy-11150R PCDHGA9原鈣粘蛋白γA9抗體
xy-11151R PCDHGA11原鈣粘蛋白γA11抗體
xy-11152R PCDHGA12原鈣粘蛋白γA12抗體
xy-11153R PCDHB12原鈣粘蛋白β12抗體
xy-11154R PCDHB3原鈣粘蛋白β3抗體
xy-11155R PCDHGB1原鈣粘蛋白γB1抗體
xy-11156R PCDHGB2原鈣粘蛋白γB2抗體
xy-11157R PCDHGB4原鈣粘蛋白γB4抗體
xy-11158R PCDHGB6原鈣粘蛋白γB6抗體
xy-11159R PCDHGC3原鈣粘蛋白γC3抗體
xy-11160R PCDHGC4原鈣粘蛋白γC4抗體
xy-11327R PTP zeta磷酸蛋白聚糖PTPRZ抗體
xy-11328R PTPN5/STEP神經(jīng)特異蛋白酪氨酸磷酸酶N5抗體
xy-8688R POD1足細(xì)胞表達(dá)蛋白/轉(zhuǎn)錄因子21抗體
xy-0183R progesterone receptor孕激素受體抗體
xy-11354R Piccolo神經(jīng)細(xì)胞突觸蛋白PCLO抗體
xy-6456R PEN2 早老素γ分泌酶2抗體
xy-8337R PANK1泛酸激酶1抗體
xy-8338R PANK2泛酸激酶2抗體
xy-8339R PANK3泛酸激酶3抗體
xy-8325R PA26p53調(diào)控PA26核蛋白抗體