Antibody data

Product number
BS5625
Provider
Bioworld Technology, Inc
Product name
Atrophin 1 polyclonal antibody
Provider product page
Bioworld Technology, Inc - BS5625
Antibody type
Polyclonal
Antigen
Synthetic peptide, corresponding to amino acids 135-180 of Human Atrophin-1.
Description
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£ª body, often resulting in dementia, epilepsy and cerebellar ataxia. HRS is characterized by the degeneration of multiple systems, resembling that of DRPLA or Huntington£ªs disease.
Reactivity
Human, Mouse, Rat
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Provider Type Product Number
- No reagents -