ATXN1, Mouse, Clone: 4C5, Abnova™

Catalog No.	Quantity
89-123-284	100 μg

Catalog No. 89-123-284 Supplier Abnova Corporation Supplier No. H00006310M02

Description

Mouse monoclonal antibody raised against a partial recombinant ATXN1.

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 41-81 CAG repeats, compared to 6-39 in the normal allele. At least two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq]

Sequence: KGSIIQLANGELKKVEDLKTEDFIQSAEISNDLKIDSSTVERIEDSHSPGVAVIQFAVGEHRAQVSVEVLVEYPFFVFGQGWSSCCPERTSQLFDLPCSK

Specifications

• Antigen: ATXN1
• Applications: ELISA, Western Blot
• Classification: Monoclonal
• Clone: 4C5
• Conjugate: Unconjugated
• Description: Mouse monoclonal antibody raised against a partial recombinant ATXN1.
• Formulation: PBS with no preservative; pH 7.4
• Gene: ATXN1
• Gene Accession No.: NM_000332
• Gene Alias: ATX1/D6S504E/SCA1
• Gene Symbols: ATXN1
• Host Species: Mouse
• Immunogen: ATXN1 (NP_000323, 576 a.a. ∼ 675 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
• Purification Method: Affinity chromatography
• Quantity: 100 μg
• Regulatory Status: RUO
• Primary or Secondary: Primary
• Gene ID (Entrez): 6310
• Target Species: Human
• Content And Storage: Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
• Product Type: Antibody
• Form: Liquid
• Isotype: IgG2b κ