Ataxin 1 (ATXN1) (pThr236) antibody

Antigen: Ataxin 1 (ATXN1)

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

Binding Site: pThr236

Clonality: Polyclonal

Host: Rabbit

Reactivity: Human

Conjugate: Un-conjugated

Application: Dot Blot (Dot), ELISA

Certificates: ISO 9001:2008

Catalog no.: ABIN389951

Additional Information

Alternative name: ATXN1

Gene ID: 6310

UniProt: NP_000323

Immunogen: This Phospho-ATXN1-pT236 antibody is generated from rabbits immunized with a KLH conjugated synthetic phosphopeptide between 219~249 amino acids surrounding T236 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 Phospho-ATXN1-pT236 antibody is generated from rabbits immunized with a KLH conjugated synthetic phosphopeptide between 219~249 amino acids surrounding T236 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. 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.

Application Details

Application Notes: The suggested dilution is: ELISA ~~ 1:1,000 Dot Blot ~~ 1:500

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 two-step phosphospecific 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, Phospho-specific antibodies

Restrictions: For Research Use only

Images

Dot blot analysis of anti-Phospho-ATXN1-pT236 Antibody (ABIN389951) on nitrocellulose membrane. 50ng of Phospho-peptide or Non Phospho-peptide per dot were adsorbed. Antibody working concentrations are 0.5ug per ml.

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).