Ataxin 1 (ATXN1) (Ser776) antibody

Antigen: Ataxin 1 (ATXN1)

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

Binding Site: Ser776

Clonality: Polyclonal

Host: Rabbit

Reactivity: Human

Conjugate: Un-conjugated

Application: Western Blotting (WB), ELISA

Certificates: ISO 9001:2008

Catalog no.: ABIN389309

Additional Information

Alternative name: ATXN1

Gene ID: 6310

UniProt: P54253

Immunogen: This ATXN1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 761~791 amino acids surrounding S776 of human ATXN1.

Isotype: Ig

Description: Other names: Ataxin-1, Spinocerebellar ataxia type 1 protein, ATXN1, ATX1, SCA1

Characteristics: Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Specificity: This ATXN1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 761~791 amino acids surrounding S776 of human ATXN1.

Molecular Weight: 86923 DA

Comments:

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.

Application Details

Application Notes: The suggested dilution is: ELISA ~~ 1:1,000 Western blotting~~ 1:50~100

Concentration: 0.25 mg/ml

Purification: Purified

Buffer: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Storage: Maintain refrigerated at 2-8 deg C for up to 6 months. For long term storage store at -20 deg C in small aliquots to prevent freeze-thaw cycles

Research Area: Stem Cells

Restrictions: For Research Use only

Images

Western blot analysis of anti-ATXN1 Antibody (S776) (ABIN389309) in CEM cell line lysates (35ug/lane). ATXN1 (arrow) was detected using the purified Pab.

Confocal immunofluorescent analysis of ATXN1 Antibody (S776)(ABIN389309) with hela cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).

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).

Hong, Lee, Cho et al.: "UbcH6 interacts with and ubiquitinates the SCA1 gene product ataxin-1." in: Biochemical and biophysical research communications, Vol. 371, Issue 2, pp. 256-60, 2008 (PubMed).