Invitrogen™ Human Ataxin 1 (aa 229-355) Control Fragment Recombinant Protein

Product Code. 30212312

Brand: Invitrogen™ RP89552

Description

Recombinant Protein

Highest antigen sequence indentity to the following orthologs: Mouse (81%), Rat (81%). This recombinant protein control fragment may be used for blocking experiments. In IHC/ICC and WB experiments, we recommend a 100x molar excess of the protein fragment control based on the concentration and the molecular weight. Pre-incubate the antibody-protein control fragment mixture for 30 min at room temperature.

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, and is associated with spinocerebellar ataxia type 1 (SCA1). At least two transcript variants encoding the same protein have been found for this gene.

Specifications

• Accession Number: P54253
• Concentration: ≥5.0 mg/mL
• For Use With (Application): Blocking Assay, Control
• Formulation: 1 M urea, PBS with no preservative; pH 7.4
• Gene ID (Entrez): 6310
• Name: Human Ataxin 1 (aa 229-355) Control Fragment
• Quantity: 100 μL
• Regulatory Status: RUO
• Gene Alias: 2900016G23Rik; alternative ataxin1; ataxin 1; Ataxin1; ataxin-1; At x 1; Atx-1; Atx-1-PB; Atxn1; C85907; CG4547; CG4547-PB; D6S504E; dAtx-1; Dmel\CG4547; Dmel_CG4547; ENSMUSG00000074917; Gm10786; OTTHUMP00000016065; SCA1; spinocerebellar ataxia 1; spinocerebellar ataxia 1 homolog; spinocerebellar ataxia type 1 protein; Spinocerebellar ataxia type 1 protein homolog
• Common Name: Ataxin 1
• Gene Symbol: ATXN1
• Conjugate: Unconjugated
• Species: Human
• Recombinant: Recombinant
• Protein Tag: His-ABP-tag
• Sequence: RAPGLITPGSPPPAQQNQYVHISSSPQNTGRTASPPAIPVHLHPHQTMIPHTLTLGPPSQVVMQYADSGSHFVPREATKKAESSRLQQAIQAKEVLNGEMEKSRRYGAPSSADLGLGKAGGKSVPHP
• Content And Storage: -20°C, Avoid Freeze/Thaw Cycles
• Expression System: E. coli
• Form: Liquid
• Purity or Quality Grade: >80% by SDS-PAGE and Coomassie blue staining