ATXN1 Antibody | 22-058

ATXN1 Antibody | 22-058 | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human, Mouse, Rat

Homology: N/A

Immunogen: Recombinant fusion protein containing a sequence corresponding to amino acids 586-815 of human ATXN1 (NP_001121636.1) .

Research Area: Neuroscience, Signal Transduction

Tested Application: WB, IF

Application: WB: 1:500 - 1:2000
IF: 1:10 - 1:100

Specificiy: N/A

Positive Control 1: A-431

Positive Control 2: 293T

Positive Control 3: HT-29

Positive Control 4: NCI-H460

Positive Control 5: SKOV3

Positive Control 6: Mouse brain

Molecular Weight: Observed: 87kDa

Validation: N/A

Isoform: N/A

Purification: Affinity purification

Clonality: Polyclonal

Clone: N/A

Isotype: IgG

Conjugate: Unconjugated

Physical State: Liquid

Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Concentration: N/A

Storage Condition: Store at -20˚C. Avoid freeze / thaw cycles.

Alternate Name: ATXN1, spinocerebellar ataxia 1 (olivopontocerebellar ataxia 1, autosomal dominant, ataxin 1) , olivopontocerebellar ataxia 1, OTTHUMP00000039306, OTTHUMP00000016065, SCA1, D6S504E, ATX1, ataxin 1, ATXN1

User Note: Optimal dilutions for each application to be determined by the researcher.

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 40-83 CAG repeats, compared to 6-39 in the normal allele, and is associated with spinocerebellar ataxia type 1 (SCA1) . At least two transcript variants encoding the same protein have been found for this gene.