Technical Data
A4159-05A
ATX2 (Ataxin 2, ATX-2, ATXN2, ATXN-2, SCA2, SCA-2, Spinocerebellar Ataxia 2, TNRC13, TNRC-13, Trinucleotide Repeat-containing Gene 13 Protein)
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 12, and it has been determined that the diseased allele contains 36-52 CAG repeats, compared to 22-23 in the normal allele. A potential transcript variant, missing an internal coding exon, has been described; however, its full-length nature is not certain.

Applications:
Suitable for use in Western Blot and Immunoprecipitation. Other applications not tested.

Recommended Dilution:
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot Store at -20C. Aliquots are stable for at least 12 months at -20C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
TypeIsotypeCloneGrade
PabAffinity Purified
SizeStorageShippingSourceHost
100ul-20CBlue IceHumanRabbit
Concentration:
~0.2mg/ml
Immunogen:
Antibody was affinity purified using an epitope specific to ATX2/SCA2 immobilized on solid support.The epitope recognized by this antibody maps to a region between residue 1262 and 1312 of human ataxin 2 (spinocerebellar ataxia 2) using the numbering give
Purity:
Purified by immunoaffinity chromatography.
Form
Tris-buffered Saline 0.1% BSA
Specificity:
Species Crossreactivity: Human.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.