产品名称：Anti-ATXN10/FITC 抗体

Anti-ATXN10/FITC 抗体

产品编号YB-11806R-FITC

英文名称Anti-ATXN10/FITC

中文名称FITC标记的脊髓小脑共济失调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/FITC 抗体

说 明 书100ul  

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    状Lyophilized or Liquid

浓    度1mg/ml

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Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

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.

Anti-ATXN10/FITC 抗体

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.Anti-ATXN10/FITC 抗体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).

Anti-ATXN10/FITC 抗体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:

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.