Ataxin 1 (ATXN1) antibody

Antigen: Ataxin 1 (ATXN1)

Synonyms: ATX1, SCA1, D6S504E, ATXN1, atxn1, Atx1, Sca1, C85907, Ataxin-1, CG4547, dAtx-1, dAtx1, DmelCG4547, sca1

Clonality: Monoclonal (2F5)

Host: Mouse

Reactivity: Human

Conjugate: Un-conjugated

Application: ELISA, Immunofluorescence (IF), Immunohistochemistry (IHC), Flow Cytometry (FACS), Western Blotting (WB)

Catalog no.: ABIN968970

Additional Information

Alternative name: ATXN1

Gene ID: 6310

Immunogen: Purified recombinant fragment of human ATXN1 expressed in E. Coli.

Format: Liquid

Isotype: IgG1

Clone: 2F5

Description: 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 41-81 CAG repeats, compared to 6-39 in the normal allele. At least two transcript variants encoding the same protein have been found for this gene.Tissue specificity: Widely expressed throughout the body.
Synonyms: ATX1, SCA1, D6S504E, ATXN1

Molecular Weight: 87kDa

Application Details

Application Notes: Western Blotting: 1/500 - 1/2000.
Immunohistochemistry: 1/200 - 1/1000.
Immunofluorescence: 1/200 - 1/1000.
Flow cytometry: 1/200 - 1/400.
ELISA: Propose dilution 1/10000.
Not yet tested in other applications.
Determining optimal working dilutions by titration test.

Purification: Ascites

Buffer: Ascitic fluid containing 0.03% sodium azide.

Preservative: 0.03% sodium azide

Storage: Store at 4℃, for long term storage, store at -20℃.

Restrictions: For Research Use only

Publications

Product: Lim, Crespo-Barreto, Jafar-Nejad et al.: "Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1." in: Nature, Vol. 452, Issue 7188, pp. 713-8, 2008 (PubMed).