Anti-ATXN3L antibody (bs-4807R)

Anti-ATXN3L antibody

DATASHEET

Host:Rabbit

Target Protein:ATXN3L

IR:Immunogen Range:251-355/355

Clonality:Polyclonal

Isotype:IgG

Entrez Gene:25814

Swiss Prot:Q9H3M9

Source:KLH conjugated synthetic peptide derived from human ATXN3L:251-355/355 

Purification:affinity purified by Protein A

Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

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. 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.

Size:100ul

Concentration:1mg/ml

Applications:WB(1:500-2000)
IHC-P(1:100-500)
IHC-F(1:100-500)
IF(1:50-200)

Cross Reactive Species:Human
.

For research use only. Not intended for diagnostic or therapeutic use.

VALIDATION IMAGES

Sample:
Hela(Human) Cell Lysate at 30 ug
MCF-7(Human) Cell Lysate at 30 ug
293T(Human) Cell Lysate at 30 ug
Primary: Anti-ATXN3L (bs-4807R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 41 kD
Observed band size: 41 kD