英文名稱 ATXN10
中文名稱 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.
脊髓小腦共濟失調10抗體 
說 明 書 0.2ml  
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 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）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 53kDa
細胞定位 細胞漿 
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
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 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.

Database links:

Entrez Gene: 25814 Human

Entrez Gene: 54138 Mouse

Omim: 611150 Human

SwissProt: Q9UBB4 Human

SwissProt: P28658 Mouse

SwissProt: Q5RE06 Orangutan

Unigene: 475125 Human

Unigene: 248906 Mouse

Important Note:
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