MAB5360
Anti-Spinocerebellar Ataxia Type 3 Antibody, clone 1H9
ascites fluid, clone 1H9, Chemicon®
Synonyme(s) :
Ataxin-3, josephin
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About This Item
Code UNSPSC :
12352203
eCl@ss :
32160702
Nomenclature NACRES :
NA.41
Produits recommandés
Source biologique
mouse
Niveau de qualité
Forme d'anticorps
ascites fluid
Type de produit anticorps
primary antibodies
Clone
1H9, monoclonal
Espèces réactives
rat, human, monkey, mouse
Fabricant/nom de marque
Chemicon®
Technique(s)
ELISA: suitable
immunocytochemistry: suitable
immunohistochemistry: suitable
immunoprecipitation (IP): suitable
western blot: suitable
Isotype
IgG1
Numéro d'accès NCBI
Numéro d'accès UniProt
Conditions d'expédition
dry ice
Modification post-traductionnelle de la cible
unmodified
Informations sur le gène
human ... ATXN3(4287)
Description générale
Spinocerebellar ataxia (SCA) is a genetic disease with multiple types, each of which could be considered a disease in its own right.
Spinocerebellar ataxia (SCA) is one of a group of genetic disorders characterized by slowly progressive incoordination of gait and often associated with poor coordination of hands, speech, and eye movements. Frequently, atrophy of the cerebellum occurs. The first ataxia gene was identified in 1993 for a dominantly inherited type called “Spinocerebellar ataxia type 1" (SCA1). Subsequently, as additional dominant genes were found they were called SCA2, SCA3, etc. Usually, the "type" number of "SCA" refers to the order in which the gene was found. At this time, there are at least 29 different gene mutations which have been found.
Spinocerebellar ataxia (SCA) is one of a group of genetic disorders characterized by slowly progressive incoordination of gait and often associated with poor coordination of hands, speech, and eye movements. Frequently, atrophy of the cerebellum occurs. The first ataxia gene was identified in 1993 for a dominantly inherited type called “Spinocerebellar ataxia type 1" (SCA1). Subsequently, as additional dominant genes were found they were called SCA2, SCA3, etc. Usually, the "type" number of "SCA" refers to the order in which the gene was found. At this time, there are at least 29 different gene mutations which have been found.
Spécificité
Ataxin-3. The epitope was mapped precisely at E214-L233. MAB5360 can be used to study wild type ataxin-3 and the mutant form with polyglutamine expansion found in patients affected with spinocerebellar ataxin type 3/Machado-Joseph disease (SCA3/MJD). In analysis of human tissues by Western blot, MAB5360 releaved several isoforms of ataxin-3 (presumably generated by alternative splicing, Trottier et al. 1998). The antibody detected polyglutamine aggregate (or nuclear inclusions) by IHC on SCA-3/MJD brain sections (Paulson et al. 1997).
Immunogène
Human ataxin-3 fragment from aa F112-L249 as a fusion protein
Application
Detect Spinocerebellar Ataxia Type 3 using this Anti-Spinocerebellar Ataxia Type 3 Antibody, clone 1H9 validated for use in ELISA, IC, IH, IP & WB.
Immunohistochemistry:
A 1:500-1:5000 dilution of a previous lot was used in IH.
Immunoprecipitation:
A 1:500-1:5000 dilution of a previous lot was used in IP.
ELISA:
A 1:500-1:5000 dilution of a previous lot was used in ELISA.
Immunocytochemistry:
A 1:500-1:5000 dilution of a previous lot was used in IC.
Optimal working dilutions must be determined by the end user.
A 1:500-1:5000 dilution of a previous lot was used in IH.
Immunoprecipitation:
A 1:500-1:5000 dilution of a previous lot was used in IP.
ELISA:
A 1:500-1:5000 dilution of a previous lot was used in ELISA.
Immunocytochemistry:
A 1:500-1:5000 dilution of a previous lot was used in IC.
Optimal working dilutions must be determined by the end user.
Qualité
Evaluated by Western Blot on NIH/3T3 lysates.
Western Blot Analysis:
1:500 dilution of this antibody detected SPINOCEREBELLAR ATAXIA 3 on 10 µg of NIH/3T3 lysates.
Western Blot Analysis:
1:500 dilution of this antibody detected SPINOCEREBELLAR ATAXIA 3 on 10 µg of NIH/3T3 lysates.
Description de la cible
44 kDa
Remarque sur l'analyse
Control
Human SCA-3/MJD brain sections, NIH/3T3 lysate
Human SCA-3/MJD brain sections, NIH/3T3 lysate
Autres remarques
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
Informations légales
CHEMICON is a registered trademark of Merck KGaA, Darmstadt, Germany
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Code de la classe de stockage
10 - Combustible liquids
Classe de danger pour l'eau (WGK)
nwg
Point d'éclair (°F)
Not applicable
Point d'éclair (°C)
Not applicable
Certificats d'analyse (COA)
Recherchez un Certificats d'analyse (COA) en saisissant le numéro de lot du produit. Les numéros de lot figurent sur l'étiquette du produit après les mots "Lot" ou "Batch".
Déjà en possession de ce produit ?
Retrouvez la documentation relative aux produits que vous avez récemment achetés dans la Bibliothèque de documents.
Y Trottier et al.
Neurobiology of disease, 5(5), 335-347 (1999-03-09)
Spinocerebellar ataxia type 3 or Machado-Joseph disease (SCA3/MJD) is an autosomal dominant neurodegenerative disorder caused by an unstable and expanded CAG trinucleotide repeat that leads to the expansion of a polyglutamine tract in a protein of unknown function, ataxin-3. We
Christopher P Reina et al.
Cell stress & chaperones, 17(6), 729-742 (2012-07-11)
Regulation of basal and induced levels of hsp70 is critical for cellular homeostasis. Ataxin-3 is a deubiquitinase with several cellular functions including transcriptional regulation and maintenance of protein homeostasis. While investigating potential roles of ataxin-3 in response to cellular stress
Overexpression of Mutant Ataxin-3 in Mouse Cerebellum Induces Ataxia and Cerebellar Neuropathology.
Nobrega, Clevio, et al.
Cerebellum (2012)
Allele-selective inhibition of ataxin-3 (ATX3) expression by antisense oligomers and duplex RNAs.
Hu, J; Gagnon, KT; Liu, J; Watts, JK; Syeda-Nawaz, J; Bennett, CF; Swayze, EE; Randolph et al.
Biological Chemistry null
Fiona M Menzies et al.
Brain : a journal of neurology, 133(Pt 1), 93-104 (2009-12-17)
Spinocerebellar ataxia type 3 is a neurodegenerative disorder caused by the expansion of the polyglutamine repeat region within the ataxin-3 protein. The mutant protein forms intracellular aggregates in the brain. However, the cellular mechanisms causing toxicity are still poorly understood
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