Thermo Fisher Scientific ATXN1 Monoclonal Antibody (4C5)

Applications

Western Blot (WB)

• Tested Dilution: 1–5 µg/mL

ELISA

• Tested Dilution: 10 µg/mL

Product Specifications

Product Specific Information

Sequence of this protein is as follows:
KGSIIQLANG ELKKVEDLKT EDFIQSAEIS NDLKIDSSTV ERIEDSHSPG VAVIQFAVGE HRAQVSVEVL VEYPFFVFGQ GWSSCCPERT SQLFDLPCSK

Target Information

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.
ADCAII 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 these repeats, producing an elongated polyglutamine tract in the corresponding protein.
The expanded repeats are variable and unstable, increasing in size in successive generations.

This locus has been mapped to chromosome 6, with diseased alleles containing 41–81 CAG repeats (normal: 6–39), associated with spinocerebellar ataxia type 1 (SCA1).
At least two transcript variants encoding the same protein have been found for this gene.

For Research Use Only.
Not for use in diagnostic procedures or resale without express authorization.