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Phospho-Ataxin 1Ser775 (Ser775) Rabbit anti-Human, Polyclonal, Bioss
SDP

Catalog No. 501982318
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50-198-2318 100 μL
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Catalog No. 50-198-2318 Supplier Bioss Supplier No. BS12534R
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Rabbit Polyclonal Antibody

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

Antigen Phospho-Ataxin 1Ser775 (Ser775)
Applications Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Concentration 1 μg/mL
Conjugate Unconjugated
Formulation PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
Gene ATXN1
Gene Accession No. P54253
Gene Alias 2900016G23Rik; alternative ataxin1; ataxin 1; Ataxin1; Ataxin-1; ATX1; Atx-1; Atx-1-PB; Atxn1; C85907; CG4547; CG4547 gene product from transcript CG4547-RB; CG4547-PB; D6S504E; dAtx1; 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
Gene Symbols ATXN1
Host Species Rabbit
Immunogen KLH conjugated synthetic phosphopeptide derived from human Ataxin 1 around the phosphorylation site of Ser776.
Purification Method Protein A
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 6310
Target Species Human
Content And Storage -20°C
Product Type Antibody
Form Liquid
Isotype IgG
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