小脑脊髓共济失调蛋白3抗体

参考价:¥1
供货周期: 现货
品牌: 康朗生物
规格: 0.1ml/0.2ml
货号: KL4807R
CAS号:
上海康朗生物科技有限公司
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产品详情

?小脑脊髓共济失调蛋白3抗体Quantity size:

0.2ml

Concentration:

1mg/ml   Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Background:

Defects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3) ; also known as Machado-Joseph disease (MJD). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders.?小脑脊髓共济失调蛋白3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. SCA3 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,?小脑脊髓共济失调蛋白3抗体 peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.

Also known as:

?小脑脊髓共济失调蛋白3抗体ATX3L_HUMAN; ATXN3L; Machado-Joseph disease protein 1-like; MJDL; Putative ataxin-3-like protein.

Specificity:

Rabbit Polyclonal IgG, affinity purified by Protein A.

Reacts with: Human, .

Immunogen: KLH conjugated synthetic peptide derived from human ATXN3L.

Predicted Molecular Weight: 41kDa.

?小脑脊髓共济失调蛋白3抗体Storage:

Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).

Application:

WB=1:100-500  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:50-200   
Not yet tested in other applications. 
Optimal working dilutions must be determined by the end user.

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小脑脊髓共济失调蛋白3抗体信息由上海康朗生物科技有限公司为您提供,如您想了解更多关于小脑脊髓共济失调蛋白3抗体报价、型号、参数等信息, 欢迎来电或留言咨询。除供应小脑脊髓共济失调蛋白3抗体外,上海康朗生物科技有限公司还可为您提供共济失调蛋白7样2抗体、共济失调7样蛋白3B抗体等产品, 公司有专业的客户服务团队,是您值得信赖的合作伙伴。

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