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

Classification:

Primary antibody

Alternative names:

ATXN1; ATX1; SCA1; Ataxin-1; Spinocerebellar ataxia type 1 protein

Species reactivity:

Human

Host:

Mouse

Isotype:

IgG

Immunogen:

Purified recombinant fragment of human Ataxin-1 expressed in E. Coli.

Concentration:

1 mg/ml

Molecular weight:

N/A

Applications:

WB, IHC-p, IF, ICC, FCM, ELISA

Storage:

-20°C for one year

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