Ataxin 1 antibody (AA 645-815)

Catalog No. ABIN7193320

Product Details anti-Ataxin 1 Antibody

Target: Ataxin 1 (ATXN1)

Binding Specificity: AA 645-815

Reactivity: Human

Host: Mouse

Clonality: Monoclonal

Conjugate: This Ataxin 1 antibody is un-conjugated

Application: Western Blotting (WB), ELISA, Flow Cytometry (FACS)

Purpose: ATXN1 Antibody

Purification: Purified antibody

Immunogen: Purified recombinant fragment of human ATXN1 (AA: 645-815) expressed in E. Coli.

Clone: 2B8A2

Isotype: IgG1

Application Details

Application Notes:

ELISA: 1/10000

FCM: 1/200 - 1/400

Restrictions: For Research Use only

Handling

Format: Liquid

Buffer: Purified antibody in PBS with 0.05 % sodium azide.

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: 4 °C,-20 °C

Storage Comment: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.

Target Details for Ataxin 1

Target: Ataxin 1 (ATXN1)

Alternative Name: ATXN1 (ATXN1 Products)

Synonyms: ATX1 antibody, D6S504E antibody, SCA1 antibody, ATXN1 antibody, ataxin 1b antibody, atxn1 antibody, 2900016G23Rik antibody, Atx1 antibody, C85907 antibody, ENSMUSG00000074917 antibody, Gm10786 antibody, Sca1 antibody, CG4547 antibody, Dmel\\CG4547 antibody, dAtx-1 antibody, dAtx1 antibody, sca1 antibody, ataxin 1 antibody, ataxin 1b antibody, Ataxin 1 antibody, ATXN1 antibody, atxn1b antibody, Atxn1 antibody, Atx-1 antibody

Background:

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 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.

Aliases: ATX1, SCA1, D6S504E

Molecular Weight: 86.9kDa

Gene ID: 6310

UniProt: P54253

Pathways: Synaptic Membrane