Background:

Dentatorubral-pallidoluysian atrophy protein, also designated atrophin-1, interacts with several other proteins, including RERE, BAIAP2 and WWP1-3. It is highly expressed in ovary, testis, brain and prostate, but can also be detected in thymus, liver and leukocytes. Defects in ATN1, the gene encoding for the atrophin protein, can cause dentatorubral-pallidoluysian atrophy (DRPLA) or Haw River syndrome (HRS). Both disorders are dominant neurodegenerative disorders caused by an increase in the number of polyglutamine (Gln) repeats in the ATN1 gene (7-23 repeats in the normal population, 49-75 in patients affected by DRPLA or HRS). More repeats corresponds to earlier onset and more severe clinical manifestations of the diseases. DRPLA is characterized by a loss of neurons in the dentate nucleus, rubrum, globus pallidus and Luys’ body, often resulting in dementia, epilepsy and cerebellar ataxia. HRS is characterized by the degeneration of multiple systems, resembling that of DRPLA or Huntington’s disease.

Specificity:

Atrophin-1 polyclonal antibody detects endogenous levels of Atrophin-1 protein.

Alternative names:

Atrophin1; Atrophin 1; Dentatorubral-pallidoluysian atrophy protein; ATN1; D12S755E; DRPLA

Species reactivity:

Human,Mouse,Rat

Host:

Rabbit

Ig type:

Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Immunogen:

Synthetic peptide, corresponding to amino acids 135-180 of Human Atrophin-1.

Purification & Purity:

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Molecular weight:

~ 150-180 kDa

Applications:

WB ICC IP

Usage:

WB: 1:500~1:1000 ICC: 1:50~1:200 IP: 1:50~1:200

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.

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