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FITC標記的脊髓小腦共濟失調10抗體

文字:[大][中][小] 2017-5-2    瀏覽次數:1419    

                                     FITC標記的脊髓小腦共濟失調10抗體                                                                                                                                                
英文名稱Anti-ATXN10/FITC
中文名稱:FITC標記的脊髓小腦共濟失調10抗體
別    名Ataxin 10; Ataxin-10; ATX10_HUMAN; Atxn10; Brain protein E46 homolog; E46L; FLJ37990; HUMEEP; Like mouse brain protein E46; SCA10; Spinocerebellar ataxia 10; Spinocerebellar ataxia type 10 protein.  

詳細介紹:


規格:100ul 
說 明 書100ul  
研究領域細胞生物  神經生物學  
抗體來源Rabbit
克隆類型Polyclonal
交叉反應 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, 
產品應用ICC=1:50-200 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量53kDa
性    狀Lyophilized or Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human ATXN10/SCA10
亞    型IgG
純化方法affinity purified by Protein A
儲 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.

相關資料:


產品介紹background:
Spinocerebellar ataxia (SCA) is an autosomal dominant neurodegenerative disorder characterized by ataxia and selective neuronal cell loss. SCA is caused by the expansion of a translated CAG repeat, encoding a polyglutamine tract in SCA gene products, known as ataxins. The ataxin proteins are ubiquitously expressed in nervous tissue, but are primarily detected in cerebellum, brain stem and spinal cord in the central nervous system. Ataxin-10 is a cytoplasmic protein that belongs to the family of armadillo repeat proteins. A loss of ataxin-10 in primary neuronal cells causes increased apoptosis of cerebellar neurons. Ataxin-10 interacts with p110, an O-Linked beta-N-acetylglucosamine transferase, and may be important in the regulation of intracellular glycosylation levels and homeostasis in the brain. Spinocerebellar ataxia type 10 (SCA10) is an autosomal dominant disorder that causes cerebellar ataxia and seizures. SCA10 is caused by an expansion of an ATTCT pentanucleotide repeat in intron 9 of the ataxin-10 gene

Function:
Necessary for the survival of cerebellar neurons. Induces neuritogenesis by activating the Ras-MAP kinase pathway. May play a role in the maintenance of a critical intracellular glycosylation level and homeostasis.

Subunit:
Homooligomer. Interacts with OGT. Interacts with GNB2. Interacts with IQCB1.

Subcellular Location:
Cytoplasm, perinuclear region.

Tissue Specificity:
Expressed in the central nervous system.

DISEASE:
Defects in ATXN10 are the cause of spinocerebellar ataxia type 10 (SCA10) [MIM:603516]. 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 degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA10 is an autosomal dominant cerebellar ataxia (ADCA).

Similarity:
Belongs to the ataxin-10 family.

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