ATXN3L Polyclonal Antibody-BS65897

ATXN3L Polyclonal Antibody

Sizes: 50µl, 100µl

Catalogue Numbers: BS65897-50, BS65897-100

Product: 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Swiss-Prot: Q9H3M9

Host: Rabbit

Reactivity: Human,

Applications: WB

All Applications: WB=1:500-2000

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.

Purification and Purity: affinity purified by Protein A

Storage and Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Specificity: ATXN3L Polyclonal Antibody detects endogenous levels of ATXN3L protein.

Bioworld Molecular Weight: 41 kD

Note: For research use only, not for use in diagnostic procedure.

Extra Notes: Primary: Anti-ATXN3L at 1/1000 dilution

Alternative Name: ATX3L_HUMAN; ATXN3L; Machado-Joseph disease protein 1-like; MJDL; Putative ataxin-3-like protein.

Immunogen: KLH conjugated synthetic peptide derived from human ATXN3L

Conjugate: Unconjugated

Modification: Unmodified