供货周期: | 现货 |
品牌: | 康朗生物 |
规格: | 0.1ml/0.2ml |
货号: | KL4807R |
CAS号: |
?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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