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ITP0536

ITP0536
  • Gene/Protein: ATXN1
  • Product Description: Immunotag™ Ataxin-1 (phospho Ser776) Polyclonal Antibody
357.0000
Price in reward points: 357

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Immunotag™ Ataxin-1 (phospho Ser776) Polyclonal Antibody
Antibody Specification
Datasheet
Target Protein Ataxin-1 (Ser776)
Clonality Polyclonal
Storage/Stability -20°C/1 year
Application WB,IHC-p,IF,ELISA
Recommended Dilution Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/10000. Not yet tested in other applications.
Concentration 1 mg/ml
Reactive Species Human,Mouse
Host Species Rabbit
Immunogen The antiserum was produced against synthesized peptide derived from human Ataxin 1 around the phosphorylation site of Ser776. AA range:742-791
Specificity Phospho-Ataxin-1 (S776) Polyclonal Antibody detects endogenous levels of Ataxin-1 protein only when phosphorylated at S776.
Purification The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen
Form Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Gene Name ATXN1
Accession No. P54253 P54254
Alternate Names ATXN1; ATX1; SCA1; Ataxin-1; Spinocerebellar ataxia type 1 protein
Description ataxin 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
Protein Expression Brain,Cerebellum,Skeletal muscle,
Subcellular Localization nucleus,nucleoplasm,cytoplasm,nuclear matrix,nuclear RNA export factor complex,nuclear inclusion body,intracellular membrane-bounded organelle,
Protein Function 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 onset and more severe clinical manifestations of the disease.,domain:The AXH domain is required for interaction with CIC.,function:Binds RNA in vitro. May be involved in RNA metabolism. The expansion of the polyglutamine tract may alter this function.,miscellaneous:The self-association seems to be necessary to form nuclear aggregates.,online information:Ataxin-1 entry,polymorphism:The poly-Gln region of ATXN1 is highly polymorphic (4 to 39 repeats) in the normal population and is expanded to about 40-83 repeats in spinocerebellar ataxia 1 (SCA1) patients.,similarity:Belongs to the ATXN1 family.,similarity:Contains 1 AXH domain.,subcellular location:Colocalizes with USP7 in the nucleus.,subunit:Interacts with CIC (By similarity). Interacts with ANP32A, PQBP1, UBIN, ATXN1L, USP7 and ZNF804A.,tissue specificity:Widely expressed throughout the body.,
Usage For Research Use Only! Not for diagnostic or therapeutic procedures.
Material Safety Data Sheet
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