Atrophin 1 Rabbit anti-Human, Polyclonal, Bethyl Laboratories

Rabbit Polyclonal Antibody

Supplier:  Bethyl Laboratories, Inc A300753AT

Catalog No. 50-155-8370

Description

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
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: ATN1
• Gene Alias: Atrophin1, Atrophin 1, Dentatorubral-pallidoluysian atrophy protein, ATN1, D12S755E, DRPLA
• Host Species: Rabbit
• Purification Method: Antigen affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 1822
• Content And Storage: 4° C
• Form: Liquid

• Applications: Immunoprecipitation, Western Blot
• Concentration: 1 mg/ml
• Formulation: phosphate, tris citrate with 0.09% sodium azide; pH 7-8
• Gene Accession No.: P54259
• Gene Symbols: ATN1
• Immunogen: Between 1150 and C-term
• Quantity: 10 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG