Anti-ATXN10抗体Spinocerebellar ataxia （SCA） is an autosomal dominant neurodegenerative disorder characterized by ataxia and selective neuronal

产品编号 yb-11806R
英文名称 Anti-ATXN10抗体
中文名称 脊髓小脑共济失调10抗体
别    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.
 Anti-ATXN10抗体   
说 明 书 0.1ml  0.2ml  
研究领域 细胞生物  神经生物学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, 
产品应用 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 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 53kDa
细胞定位 细胞浆 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human ATXN10/SCA10
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
 
PubMed PubMed
产品介绍 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.
 
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.