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Ataxin

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Chromatin-binding factor that repress Notch signaling in the absence of Notch intracellular domain by acting as a CBF1 corepressor. Binds to the HEY promoter and might assist, along with NCOR2, RBPJ-mediated repression (By similarity). May be involved in RNA metabolism.

Below are the list of possible Ataxin products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

Ataxin-1

 Ataxin-1 ELISA Kit
 Ataxin-1 Recombinant
 Ataxin-1 Antibody
Also known as Ataxin-1 (Spinocerebellar ataxia type 1 protein homolog).
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
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tract may alter this function.

Protein type: Nuclear export; RNA-binding

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

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

Biological Process: adult locomotory behavior; alveolus development; negative regulation of insulin-like growth factor receptor signaling pathway; negative regulation of phosphorylation; negative regulation of transcription from RNA polymerase II promoter; negative regulation of transcription, DNA-dependent; nuclear export; positive regulation of transcription from RNA polymerase II promoter; regulation of excitatory postsynaptic membrane potential; visual learning
 Atxn1 ELISA Kit
 Atxn1 Recombinant
 Atxn1 Antibody
 Sca1 ELISA Kit
 Sca1 Recombinant
 Sca1 Antibody
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Ataxin-10

 Ataxin-10 ELISA Kit
 Ataxin-10 Recombinant
 Ataxin-10 Antibody
Also known as Ataxin-10 (Brain protein E46 homolog) (Spinocerebellar ataxia type 10 protein).
ataxin-10: Necessary for the survival of cerebellar neurons. Induces neuritogenesis by activating the Ras-MAP kinase pathway. May play a role in the maintenance of a critical intracellular glycosylation level and homeostasis. Defects in ATXN10 are the cause of spinocerebellar ataxia type 10 (SCA10). Sp
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inocerebellar 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 degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA10 is an autosomal dominant cerebellar ataxia (ADCA). Defects in ATXN1 may be a cause of nephronophthisis a chronic tubulo-interstitial nephropathy taht leads to anemia, polyuria, polydipsia, isosthenuria and death in uremia. Belongs to the ataxin-10 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Cell development/differentiation

Chromosomal Location of Human Ortholog: 22q13.31

Cellular Component: cell soma; cytoplasm; dendrite; extracellular space; membrane; perinuclear region of cytoplasm; plasma membrane

Molecular Function: protein binding

Biological Process: nervous system development; neurite development

Disease: Spinocerebellar Ataxia 10
 ATXN10 ELISA Kit
 ATXN10 Recombinant
 ATXN10 Antibody
 SCA10 ELISA Kit
 SCA10 Recombinant
 SCA10 Antibody
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Ataxin-10 homolog

 Ataxin-10 homolog ELISA Kit
 Ataxin-10 homolog Recombinant
 Ataxin-10 homolog Antibody
 atxn10 ELISA Kit
 atxn10 Recombinant
 atxn10 Antibody
 DDB_G0268880 ELISA Kit
 DDB_G0268880 Recombinant
 DDB_G0268880 Antibody
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Ataxin-1-like

 Ataxin-1-like ELISA Kit
 Ataxin-1-like Recombinant
 Ataxin-1-like Antibody
Also known as Ataxin-1-like (Brother of ataxin-1).
ataxin-1L: Chromatin-binding factor that repress Notch signaling in the absence of Notch intracellular domain by acting as a CBF1 corepressor. Binds to the HEY promoter and might assist, along with NCOR2, RBPJ-mediated repression. Can suppress ATXN1 cytotoxicity in spinocerebellar ataxia type 1 (SCA1). Belongs to the ATXN1 family.

Cellul
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ar Component: nucleolus; nucleoplasm; nucleus

Molecular Function: protein binding

Biological Process: alveolus development; extracellular matrix organization and biogenesis; negative regulation of transcription from RNA polymerase II promoter
 Atxn1l ELISA Kit
 Atxn1l Recombinant
 Atxn1l Antibody
 Boat ELISA Kit
 Boat Recombinant
 Boat Antibody
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Ataxin-2

 Ataxin-2 ELISA Kit
 Ataxin-2 Recombinant
 Ataxin-2 Antibody
Also known as Ataxin-2 (Spinocerebellar ataxia type 2 protein homolog).
ataxin-2: Involved in EGFR trafficking, acting as negative regulator of endocytic EGFR internalization at the plasma membrane. Defects in ATXN2 are the cause of spinocerebellar ataxia type 2 (SCA2); also known as olivopontocerebellar atrophy II (OPCA II or OPCA2). 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 in
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volvement of the brainstem and spinal cord. SCA2 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. SCA2 is characterized by hyporeflexia, myoclonus and action tremor and dopamine-responsive parkinsonism. SCA2 is caused by expansion of a CAG repeat resulting in about 36 to 52 repeats in some patients. Longer expansions result in earlier the expansion, onset of the disease. Defects in ATXN2 are a cause of susceptibility to amyotrophic lateral sclerosis type 13 (ALS13). It is a neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases. An increased risk for developing amyotrophic lateral sclerosis is seems to be conferred by CAG repeat intermediate expansions greater than 23 but below the threshold for developing spinocerebellar ataxia. Belongs to the ataxin-2 family. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: RNA-binding; Translation

Cellular Component: cytoplasm; Golgi apparatus; membrane; nucleoplasm; perinuclear region of cytoplasm; polysome; ribonucleoprotein complex; stress granule; trans-Golgi network

Molecular Function: DBD domain binding; epidermal growth factor receptor binding; protein C-terminus binding

Biological Process: cerebellar Purkinje cell differentiation; cytoplasmic mRNA processing body assembly; homeostasis of number of cells; negative regulation of multicellular organism growth; negative regulation of receptor internalization; neurite morphogenesis; neuromuscular process; stress granule assembly
 Atxn2 ELISA Kit
 Atxn2 Recombinant
 Atxn2 Antibody
 Atx2 ELISA Kit
 Atx2 Recombinant
 Atx2 Antibody
 Sca2 ELISA Kit
 Sca2 Recombinant
 Sca2 Antibody
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Ataxin-2 homolog

 Ataxin-2 homolog ELISA Kit
 Ataxin-2 homolog Recombinant
 Ataxin-2 homolog Antibody
 atxn2 ELISA Kit
 atxn2 Recombinant
 atxn2 Antibody
 DDB_G0269682 ELISA Kit
 DDB_G0269682 Recombinant
 DDB_G0269682 Antibody
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Ataxin-2-like protein

 Ataxin-2-like protein ELISA Kit
 Ataxin-2-like protein Recombinant
 Ataxin-2-like protein Antibody
ataxin-2L: an ataxin type 2 related protein of unknown function. This protein is a member of the spinocerebellar ataxia (SCAs) family, which is associated with a complex group of neurodegenerative disorders. Several alternatively spliced transcripts encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

Protein type: Membrane protein, peripheral; RNA-bindi
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ng

Cellular Component: cell-cell adherens junction; cytoplasm; membrane; nuclear speck; stress granule

Biological Process: stress granule assembly
 Atxn2l ELISA Kit
 Atxn2l Recombinant
 Atxn2l Antibody
 A2lp ELISA Kit
 A2lp Recombinant
 A2lp Antibody
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Ataxin-3

 Ataxin-3 ELISA Kit
 Ataxin-3 Recombinant
 Ataxin-3 Antibody
Also known as Ataxin-3 (Machado-Joseph disease protein 1 homolog).
Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins. Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription ().
 ATXN3 ELISA Kit
 ATXN3 Recombinant
 ATXN3 Antibody
 MDJ1 ELISA Kit
 MDJ1 Recombinant
 MDJ1 Antibody
 MJD ELISA Kit
 MJD Recombinant
 MJD Antibody
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Ataxin-3 homolog

 Ataxin-3 homolog ELISA Kit
 Ataxin-3 homolog Recombinant
 Ataxin-3 homolog Antibody
Also known as Ataxin-3 homolog (Machado-Joseph disease-like protein).
Interacts with key regulators of transcription and represses transcription. Acts as a histone-binding protein that regulates transcription. Acts as a deubiquitinating enzyme ().
 atx-3 ELISA Kit
 atx-3 Recombinant
 atx-3 Antibody
 F28F8.6 ELISA Kit
 F28F8.6 Recombinant
 F28F8.6 Antibody
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Ataxin-3-like protein

 Ataxin-3-like protein ELISA Kit
 Ataxin-3-like protein Recombinant
 Ataxin-3-like protein Antibody
Also known as Ataxin-3-like protein (Machado-Joseph disease protein 1-like).
ataxin-3L: Deubiquitinating enzyme that cleaves both 'Lys-48'- linked and 'Lys-63'-linked poly-ubiquitin chains (in vitro).

Protein type: EC 3.4.19.12; Protease; Ubiquitin-specific protease

Chromosomal Location of Human Ortholog: Xp22.2

Molecular Function: ubiquitin-specific protease activity

Biological Process: protein deubiquitination
 ATXN3L ELISA Kit
 ATXN3L Recombinant
 ATXN3L Antibody
 ATX3L ELISA Kit
 ATX3L Recombinant
 ATX3L Antibody
 MJDL ELISA Kit
 MJDL Recombinant
 MJDL Antibody
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Ataxin-7

 Ataxin-7 ELISA Kit
 Ataxin-7 Recombinant
 Ataxin-7 Antibody
Also known as Ataxin-7 (Spinocerebellar ataxia type 7 protein homolog).
ataxin-7: Acts as component of the STAGA transcription coactivator-HAT complex. Mediates the interaction of STAGA complex with the CRX and is involved in CRX-dependent gene activation. Necessary for microtubule cytoskeleton stabilization. Defects in ATXN7 are the cause of spinocerebellar ataxia type 7 (SCA7); also known as
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olivopontocerebellar atrophy III (OPCA III or OPCA3) or olivopontocerebellar atrophy with retinal degeneration. 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 degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA7 belongs to the autosomal dominant cerebellar ataxias type II (ADCA II) which are characterized by cerebellar ataxia with retinal degeneration and pigmentary macular dystrophy. Belongs to the ataxin-7 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Nucleolus; Transcription regulation

Cellular Component: cytoplasm; microtubule cytoskeleton; nucleoplasm; nucleus

Molecular Function: chromatin binding; protein binding

Biological Process: histone deubiquitination; microtubule cytoskeleton organization and biogenesis; negative regulation of insulin-like growth factor receptor signaling pathway; negative regulation of phosphorylation; positive regulation of transcription from RNA polymerase II promoter
 Atxn7 ELISA Kit
 Atxn7 Recombinant
 Atxn7 Antibody
 Sca7 ELISA Kit
 Sca7 Recombinant
 Sca7 Antibody
Table BarTOPTable Bar
 

Ataxin-7-like protein 1

 Ataxin-7-like protein 1 ELISA Kit
 Ataxin-7-like protein 1 Recombinant
 Ataxin-7-like protein 1 Antibody
Also known as Ataxin-7-like protein 1 (Ataxin-7-like protein 4).
 ATXN7L1 ELISA Kit
 ATXN7L1 Recombinant
 ATXN7L1 Antibody
 ATXN7L4 ELISA Kit
 ATXN7L4 Recombinant
 ATXN7L4 Antibody
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Ataxin-7-like protein 2

 Ataxin-7-like protein 2 ELISA Kit
 Ataxin-7-like protein 2 Recombinant
 Ataxin-7-like protein 2 Antibody
ataxin-7L2: Belongs to the ataxin-7 family.

Protein type: Unknown function

Chromosomal Location of Human Ortholog: 1p13.3
 ATXN7L2 ELISA Kit
 ATXN7L2 Recombinant
 ATXN7L2 Antibody
Table BarTOPTable Bar
 

Ataxin-7-like protein 3

 Ataxin-7-like protein 3 ELISA Kit
 Ataxin-7-like protein 3 Recombinant
 Ataxin-7-like protein 3 Antibody
Also known as Ataxin-7-like protein 3 (SAGA-associated factor 11 homolog).
ataxin-7L3: Component of the transcription regulatory histone acetylation (HAT) complex SAGA, a multiprotein complex that activates transcription by remodeling chromatin and mediating histone acetylation and deubiquitination. Within the SAGA complex, participates in a subcomplex that specifically deubiquitinates both his
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tones H2A and H2B. The SAGA complex is recruited to specific gene promoters by activators such as MYC, where it is required for transcription. Required for nuclear receptor-mediated transactivation. Within the complex, it is required to recruit USP22 and ENY2 into the SAGA complex. Belongs to the SGF11 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Nuclear receptor co-regulator; Transcription, coactivator/corepressor; Ubiquitin conjugating system

Chromosomal Location of Human Ortholog: 17q21.31

Molecular Function: ligand-dependent nuclear receptor transcription coactivator activity; protein binding; transcription coactivator activity

Biological Process: histone deubiquitination; positive regulation of transcription, DNA-dependent
 ATXN7L3 ELISA Kit
 ATXN7L3 Recombinant
 ATXN7L3 Antibody
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Ataxin-8

 Ataxin-8 ELISA Kit
 Ataxin-8 Recombinant
 Ataxin-8 Antibody
Also known as Ataxin-8 (Protein 1C2).
ATXN8:

Protein type: Apoptosis

Chromosomal Location of Human Ortholog: 13q21

Disease: Spinocerebellar Ataxia 8
 ATXN8 ELISA Kit
 ATXN8 Recombinant
 ATXN8 Antibody
Table BarTOPTable Bar
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