Antibody data
- Antibody Data
- Antigen structure
- References [0]
- Comments [0]
- Validations
- Western blot [1]
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- Product number
- BS5625 - Provider product page
- Provider
- Bioworld Technology, Inc
- Product name
- Atrophin 1 polyclonal antibody
- 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.
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Supportive validation
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- Bioworld Technology, Inc (provider)
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- Experimental details
- Western blot (WB) analysis of Atrophin-1 pAb at 1:500 dilutionLane1:HEK293T cell lysateLane2:sp2/0 cell lysateLane3:H9C2 cell lysate