Product Description

ATXN3L Polyclonal Antibody

Sizes: 50μl, 100μl

Catalogue Numbers: BS65897-50, BS65897-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

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, bμlbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecμlar defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions resμlt in earlier onset and more severe clinical manifestations of the disease.

Category: Primary Antibody

Reactivity: Human

Host: Rabbit

Applications: WB

Alternate Names: ATX3L_HUMAN, ATXN3L, Machado-Joseph disease protein 1-like, MJDL, Putative ataxin-3-like protein.

Clonality: Polyclonal

Conjugate: Unconjugated

Modification: Unmodification

Immunogen: KLH conjugated synthetic peptide derived from human ATXN3L

Dilution: WB=1:500-2000

Purification: affinity purified by Protein A

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

Molecular Weight: 41 kD

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

SwissProt: Q9H3M9

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

Research Use Only