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Anti-Ataxin 3 Antibody

品牌：Antibodies

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规格：100µl

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Anti-Ataxin 3 Antibody

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Overview

Name:	Anti-Ataxin 3 Antibody See all Ataxin 3 primary antibodies
Description:	Rabbit polyclonal antibody to Ataxin 3
Applications:	WB, IHC
Reactivity:	Human
Immunogen:	Recombinant protein fragment contain a sequence corresponding to a region within amino acids 1 and 215 of Ataxin 3
Host:	Rabbit
Clonality:	Polyclonal
Conjugate:	Unconjugated
Purification:	Purified by antigen-affinity chromatography.
Formulation:	Supplied in 0.1M Tris-buffered saline with 20% Glycerol (pH7.0). 0.01% Thimerosal was added as a preservative.
Storage:	Store at -20?C for long term preservation (recommended). Store at 4?C for short term use.

Target

Function:	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. Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP: recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension. In response to misfolded substrate ubiquitination, mediates deubiquitination of monoubiquitinated STUB1/CHIP. Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription.
Tissue Specificity:	Ubiquitous.
Involvement in Disease:	Spinocerebellar ataxia 3: 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. SCA3 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. The molecular defect in SCA3 is the a CAG repeat expansion in ATX3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.
Post-Translational Modification:	Monoubiquitinated N-terminally by UBE2W, possibly leading to activate the deubiquitinating enzyme activity.
Cellular Location:	Nucleus matrix. Predominantly nuclear, but not exclusively, inner nuclear matrix.
Database Links:	Entrez Gene: 4287?Human Omim: 607047?Human SwissProt: P54252?Human Unigene: 532632?Human
Synonyms:	AT3 Antibody Ataxin 3 Antibody ataxin 3 variant h Antibody ataxin 3 variant m Antibody ataxin 3 variant ref Antibody Ataxin-3 Antibody ATX3 Antibody ATX3_HUMAN Antibody ATXN3 Antibody EC 3.4.22. Antibody JOS Antibody Josephin Antibody Machado Joseph disease Antibody Machado Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3) Antibody Machado Joseph disease protein 1 Antibody Machado-Joseph disease protein 1 Antibody Machado-Joseph disease protein 1 homolog Antibody MJD Antibody MJD gene Antibody MJD1 Antibody Olivopontocerebellar ataxia 3 Antibody OTTHUMP00000221583 Antibody OTTHUMP00000221585 Antibody OTTHUMP00000221586 Antibody OTTHUMP00000221587 Antibody OTTHUMP00000231995 Antibody OTTHUMP00000231997 Antibody Rsca3 Antibody SCA3 Antibody SCA3 gene Antibody Spinocerebellar ataxia type 3 protein Antibody
Information:	Target information shown above is from the UniProt Consortium.