Anti-ATXN3L抗体

Cat. Number:

Anti-ATXN3L抗体KL-4807R

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 Anti-ATXN3L抗体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. 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, 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:

ATX3L_HUMAN; Anti-ATXN3L抗体ATXN3L; Machado-Joseph disease protein 1-like; MJDL; Putative ataxin-3-like protein.

Specificity:

Storage:

Shipped at 4℃, Anti-ATXN3L抗体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 Anti-ATXN3L抗体dilutions must be determined by the end user.