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Atrophin 1 Rabbit anti-Human, Polyclonal, Bethyl Laboratories

Catalog No. 501558370
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50-155-8370 10 μL
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Catalog No. 50-155-8370 Supplier Bethyl Laboratories, Inc Supplier No. A300753AT
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Rabbit Polyclonal Antibody

The recommended shelf life for this product is 1 year from date of receipt. Based on 100% sequence identity, this antibody is predicted to react with Mouse, Rat and Chimpanzee

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 domit 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.

Specifications

Antigen Atrophin 1
Applications Immunoprecipitation, Western Blot
Classification Polyclonal
Concentration 1 mg/ml
Conjugate Unconjugated
Formulation phosphate, tris citrate with 0.09% sodium azide; pH 7-8
Gene ATN1
Gene Accession No. P54259
Gene Alias Atrophin1, Atrophin 1, Dentatorubral-pallidoluysian atrophy protein, ATN1, D12S755E, DRPLA
Gene Symbols ATN1
Host Species Rabbit
Immunogen Between 1150 and C-term
Purification Method Antigen affinity chromatography
Quantity 10 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 1822
Target Species Human
Content And Storage 4° C
Product Type Antibody
Form Liquid
Isotype IgG
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