Ataxin 1 (ATXN1) Rabbit Polyclonal Antibody

CAT#: TA332986

Rabbit anti-ATXN1 Polyclonal Antibody

Product Images

Western blot analysis of extracts of various cell lines, using ATXN1 antibody.

Specifications

Product Data

• Applications: ELISA, WB
• Recommended Dilution: WB,1:500 - 1:2000
  ELISA,Recommended starting concentration is 1 μg/mL. Please optimize the concentration based on your specific assay requirements.
• Reactivities: Human, Mouse, Rat
• Host: Rabbit
• Isotype: IgG
• Clonality: Polyclonal
• Formulation: Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
• Concentration: lot specific
• Purification: Affinity purification
• Conjugation: Unconjugated
• Storage: Store at -20℃. Avoid freeze / thaw cycles.
• Stability: Stable for 12 months from date of receipt.
• Predicted Protein Size: 87kDa
• Gene Name: ataxin 1
• Database Link:
  NP_000323
  Entrez Gene 20238 MouseEntrez Gene 25049 RatEntrez Gene 6310 Human
  UniProt ID P54253
• Background: The autosomal dominant cerebellar ataxias (ADCA) are a heterogeneous group of neurodegenerative disorders characterized by progressive degeneration of the cerebellum, brain stem and spinal cord. Clinically, ADCA has been divided into three groups: ADCA types I-III. ADCAI is genetically heterogeneous, with five genetic loci, designated spinocerebellar ataxia (SCA) 1, 2, 3, 4 and 6, being assigned to five different chromosomes. ADCAII, which always presents with retinal degeneration (SCA7), and ADCAIII often referred to as the `pure' cerebellar syndrome (SCA5), are most likely homogeneous disorders. Several SCA genes have been cloned and shown to contain CAG repeats in their coding regions. ADCA is caused by the expansion of the CAG repeats, producing an elongated polyglutamine tract in the corresponding protein. The expanded repeats are variable in size and unstable, usually increasing in size when transmitted to successive generations. The function of the ataxins is not known. This locus has been mapped to chromosome 6, and it has been determined that the diseased allele contains 40-83 CAG repeats, compared to 6-39 in the normal allele, and is associated with spinocerebellar ataxia type 1 (SCA1). Alternative splicing results in multiple transcript variants, with one variant encoding multiple distinct proteins, ATXN1 and Alt-ATXN1, due to the use of overlapping alternate reading frames.
• Synonyms: ATX1; D6S504E; SCA1