ATXN1 monoclonal antibody (M02), clone 4C5
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More Files
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Specification
Product Description
Mouse monoclonal antibody raised against a partial recombinant ATXN1.
Immunogen
ATXN1 (NP_000323, 576 a.a. ~ 675 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
KGSIIQLANGELKKVEDLKTEDFIQSAEISNDLKIDSSTVERIEDSHSPGVAVIQFAVGEHRAQVSVEVLVEYPFFVFGQGWSSCCPERTSQLFDLPCSK
Host
Mouse
Reactivity
Human
Interspecies Antigen Sequence
Mouse (92); Rat (100)
Isotype
IgG2b Kappa
Quality Control Testing
Antibody Reactive Against Recombinant Protein.
Western Blot detection against Immunogen (36.74 KDa) .
Storage Buffer
In 1x PBS, pH 7.4
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot (Recombinant protein)
Sandwich ELISA (Recombinant protein)
Detection limit for recombinant GST tagged ATXN1 is 3 ng/ml as a capture antibody.ELISA
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Gene Info — ATXN1
Entrez GeneID
6310GeneBank Accession#
NM_000332Protein Accession#
NP_000323Gene Name
ATXN1
Gene Alias
ATX1, D6S504E, SCA1
Gene Description
ataxin 1
Gene Ontology
HyperlinkGene Summary
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 to successive generations. The function of the ataxins is not known. This locus has been mapped to chromosome 6, and it has been determined that the diseased allele contains 41-81 CAG repeats, compared to 6-39 in the normal allele. At least two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq
Other Designations
OTTHUMP00000016065|OTTHUMP00000039306|olivopontocerebellar ataxia 1, autosomal dominant|spinocerebellar ataxia 1 (olivopontocerebellar ataxia 1, autosomal dominant, ataxin 1)
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Interactome
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Disease
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Publication Reference
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The use of scaffold-free cell sheet technique to refine mesenchymal stromal cell-based therapy for heart failure.
Takuya Narita, Yasunori Shintani, Chiho Ikebe, Masahiro Kaneko, Niall G Campbell, Steven R Coppen, Rakesh Uppal, Yoshiki Sawa, Kenta Yashiro, Ken Suzuki.
Molecular Therapy 2013 Jan; 21(4):860.
Application:Quant, Rat, Rat heart.
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The use of scaffold-free cell sheet technique to refine mesenchymal stromal cell-based therapy for heart failure.
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