ATX1, Polyclonal Antibody

Anti -ATX1 (Ataxin 1)

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 6; NC_000006.11 (16299343..16761721, complement). Location: 6p23

Polyclonal

IgG

Rabbit

Recognizes endogenous levels of total human ataxin-1 protein. Species Crossreactivity: mouse, rat and D. melanogaster

Affinity Purified
Purified by Protein A and peptide affinity chromatography.

Supplied as a liquid in 10mM sodium HEPES, pH 7.5, 150mM sodium chloride, 0.1mg/ml BSA, 50% glycerol.

May be stored at 4 degree C for short-term only. For long-term storage, aliquot and store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Small volumes of anti-ATXN1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Spinocerebellar ataxia 1 (SCA1), an autosomal dominant neurodegenerative disorder, is characterized by slurred speech, loss of limb coordination, and gait abnormalities resulting from the degeneration of cerebellar Purkinje cells and of a subset of brainstem neurons (1). Individuals with SCA1 have a highly polymorphic CAG repeat expansion encoding a polyglutamine tract in ataxin-1 (2). Akt phosphorylates ataxin-1 at Ser776, which regulates an association with 14-3-3. This interaction increases ataxin-1 stabilization and accumulation resulting in enhanced neurodegeneration (3). In addition, HSP70 controls the effect that phosphorylation has on ataxin-1 stability (4).

Antibodies; Abs to Ataxin

Western Blot (WB)

Suitable for use in Western Blot.
Dilution: Western Blot: 1:1000

86,923 Da[Similar Products]

ataxin 1

ataxin-1; OTTHUMP00000016065; spinocerebellar ataxia type 1 protein

Ataxin-1

ATX1; SCA1  [Similar Products]

ATX1_HUMAN

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. [provided by RefSeq]

ataxin-1: spinocerebellar ataxia type 1 (SCA1) protein. Expansion of CAG repeats in the SCA1 gene results in an abnormally long polyglutamine tract in the protein, causing polyglutamine-induced neurodegeneration. 14-3-3 and Akt kinase stabilize both wild-type and mutant forms of ataxin-1. The expansion of the polyglutamine tract may alter this function.

Protein type: RNA-binding; Nuclear export

Chromosomal Location of Human Ortholog: 6p23

Cellular Component: nucleoplasm; nuclear RNA export factor complex; nuclear matrix; intracellular membrane-bound organelle; cytoplasm; nucleus; nuclear inclusion body

Molecular Function: protein C-terminus binding; identical protein binding; protein binding; protein self-association; DNA binding; poly(U) binding; chromatin binding; poly(rG) binding

Biological Process: RNA processing; transcription, DNA-dependent; ***** locomotory behavior; negative regulation of insulin-like growth factor receptor signaling pathway; nuclear export; negative regulation of phosphorylation; visual learning; positive regulation of transcription from RNA polymerase II promoter; negative regulation of transcription from RNA polymerase II promoter; regulation of excitatory postsynaptic membrane potential; negative regulation of transcription, DNA-dependent; alveolus development

Disease: Spinocerebellar Ataxia 1

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