Ataxin-1 Monoclonal Antibody

Ab type

Primary antibody

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

Reactivity

Human

Clonality

Monoclonal

Host

Mouse

Isotype

IgG

Immunogen

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

Storage Stability

-20°C for one year

Concentration

1 mg/ml

MolecularWeight_Da

N/A

synonyms

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