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.

Specificity:

ATXN3L Polyclonal Antibody detects endogenous levels of ATXN3L protein.

Alternative names:

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

Species reactivity:

Human,

Host:

Rabbit

Ig type:

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

Immunogen:

KLH conjugated synthetic peptide derived from human ATXN3L

Purification & Purity:

affinity purified by Protein A

Molecular weight:

41 kD

Applications:

WB,

Usage:

WB=1:500-2000

Storage:

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

Precautions:

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

More info:

Email: info@sobekbio.com

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