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.
Applications:Suitable for use in Western Blot, Immunoprecipitation, Immunocytochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:1000Immunocytochemistry: 1:50-1:200Immunoprecipitation: 1:50-1:200Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid PBS, 0.05% sodium azide, pH 7.2.
Specificity：Recognizes endogenous levels of Atrophin-1. Species Crossreactivity: Human, mouse, rat
Isotype：IgG
Calc Applications Abbrev：IC IP WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Synthetic peptide corresponding to amino acids 135-180 of Human Atrophin-1.
