MAB5360
Anti-Spinocerebellar Ataxia Type 3 Antibody, clone 1H9
ascites fluid, clone 1H9, Chemicon®
Synonym(s):
Ataxin-3, josephin
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About This Item
UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41
Recommended Products
biological source
mouse
Quality Level
antibody form
ascites fluid
antibody product type
primary antibodies
clone
1H9, monoclonal
species reactivity
rat, human, monkey, mouse
manufacturer/tradename
Chemicon®
technique(s)
ELISA: suitable
immunocytochemistry: suitable
immunohistochemistry: suitable
immunoprecipitation (IP): suitable
western blot: suitable
isotype
IgG1
NCBI accession no.
UniProt accession no.
shipped in
dry ice
target post-translational modification
unmodified
Gene Information
human ... ATXN3(4287)
General description
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.
Specificity
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).
Immunogen
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.
Quality
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.
Target description
44 kDa
Analysis Note
Control
Human SCA-3/MJD brain sections, NIH/3T3 lysate
Human SCA-3/MJD brain sections, NIH/3T3 lysate
Other Notes
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
Legal Information
CHEMICON is a registered trademark of Merck KGaA, Darmstadt, Germany
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Storage Class Code
10 - Combustible liquids
WGK
nwg
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Certificates of Analysis (COA)
Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.
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Sandro Alves et al.
Human molecular genetics, 17(14), 2071-2083 (2008-04-04)
Machado-Joseph disease (MJD) is a fatal, dominant neurodegenerative disorder. MJD results from polyglutamine repeat expansion in the MJD-1 gene, conferring a toxic gain of function to the ataxin-3 protein. In this study, we aimed at overexpressing ataxin-3 in the rat
Huu Phuc Nguyen et al.
PloS one, 8(4), e62043-e62043 (2013-04-30)
Spinocerebellar Ataxia Type 3 (SCA3), also known as Machado-Joseph disease, is an autosomal dominantly inherited neurodegenerative disease caused by an expanded polyglutamine stretch in the ataxin-3 protein. A pathological hallmark of the disease is cerebellar and brainstem atrophy, which correlates
Rui Gao et al.
PLoS genetics, 11(1), e1004834-e1004834 (2015-01-16)
Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD), is an untreatable autosomal dominant neurodegenerative disease, and the most common such inherited ataxia worldwide. The mutation in SCA3 is the expansion of a polymorphic CAG tri-nucleotide repeat sequence
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
Nadine Griesche et al.
Frontiers in cellular neuroscience, 10, 226-226 (2016-10-25)
Expansion of CAG repeats, which code for the disease-causing polyglutamine protein, is a common feature in polyglutamine diseases. RNA-mediated mechanisms that contribute to neuropathology in polyglutamine diseases are important. RNA-toxicity describes a phenomenon by which the mutant CAG repeat RNA
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