Ataxin-1 rabbit pAb

Ataxin-1 rabbit pAb

AO-06-ES1718-50

Ataxin-1 rabbit pAb 50μL

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Antibody Product Overview

ELK.NoES1718
Product nameAtaxin-1 rabbit pAb
ReactivityHuman;Mouse
ApplicationsWB;IHC;IF;ELISA
Other nameATXN1; ATX1; SCA1; Ataxin-1; Spinocerebellar ataxia type 1 protein
Size50μL
Unit price ($)148
Human gene ID6310
Human Swiss-ProtP54253
SourceRabbit
IsotypeIgG
TargetAtaxin-1
Fields>>Notch signaling pathway;>>Spinocerebellar ataxia;>>Pathways of neurodegeneration - multiple diseases
Gene nameATXN1
Protein nameAtaxin-1
Human gene linkView Human Gene
Human Swiss linkView Human Swiss-Prot
Mouse gene ID20238
Mouse gene linkView Mouse Gene
Mouse Swiss-ProtP54254
Mouse Swiss linkView Mouse Swiss-Prot
Rat gene ID
Rat gene link
Rat Swiss-Prot
Rat Swiss link
ImmunogenThe antiserum was produced against synthesized peptide derived from human Ataxin 1. AA range:742-791
SpecificityAtaxin-1 Polyclonal Antibody detects endogenous levels of Ataxin-1 protein.
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
ClonalityPolyclonal
DilutionImmunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/5000. Not yet tested in other applications.
PurificationThe antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration1 mg/ml
Storage stability-20°C/1 year
Molecular Weight (Da)
Observed band (KD)87kD
Backgroundataxin 1(ATXN1) Homo sapiens 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
Functionalternative products:At least 2 isoforms are produced,disease:Defects in ATXN1 are the cause of spinocerebellar ataxia type 1 (SCA1) [MIM:164400]; also known as olivopontocerebellar atrophy I (OPCA I or OPCA1). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to cerebellum degeneration with variable involvement of the brainstem and spinal cord. SCA1 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. SCA1 is caused by expansion of a CAG repeat in the coding region of ATXN1. Longer expansions result in earlier
Subcellular locationCytoplasm . Nucleus . Colocalizes with USP7 in the nucleus. .
ExpressionWidely expressed throughout the body.

Additional Images

Image 1
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Western Blot analysis of various cells using Ataxin-1 Polyclonal Antibody diluted at 1:500
Image 2
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Immunofluorescence analysis of NIH/3T3 cells, using Ataxin 1 Antibody. The picture on the right is blocked with the synthesized peptide.
Image 3
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Immunohistochemistry analysis of paraffin-embedded human lung carcinoma tissue, using Ataxin 1 Antibody. The picture on the right is blocked with the synthesized peptide.
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: AO-06-ES1718-50
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Hurry! only 10 items left in stock.

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