|Western Blot (WB)||1:500-1:1000|
|Tested Species reactivity||Human, Mouse, Rat|
|Host / Isotype||Rabbit / IgG|
|Immunogen||Synthetic peptide corresponding to amino acids 135-180 of human Atrophin-1|
|Purification||Antigen affinity chromatography|
|Storage buffer||PBS, pH 7.2|
|Contains||0.05% sodium azide|
|Storage conditions||Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.|
This antibody detects endogenous protein at a molecular weight of 150-180 kDa.
Purity is >95% by SDS-PAGE.
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: ATN1; Atrophin 1; Atrophin-1; Atrophin1; D12S755E; dentatorubral pallidoluysian atrophy; Dentatorubral-pallidoluysian atrophy protein; Dentatorubral-pallidoluysian atrophy protein homolog; DRPLA
Gene Aliases: ATN1; Atr1; atrophin-1; B37; D12S755E; DRPLA; HRS; NOD
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