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Anti-Polyglutamine [5TF1-1C2]

Info

Applications ELISA IHC IF WB
Antigen/Gene or Protein Targets  Polyglutamine 
Synonyms Polyglutamine 
Reactivity N/A
Relevance Polyglutamine tract are portions of a protein consisting of a sequence of several glutamine unit. Several inheritable neurodegenerative disorders, so-called polyglutamine diseases, occur if a mutation causes a polyglutamine tract in a specific gene to become too long. Important examples of polyglutamine diseases include Huntington's disease, dentatorubralpallidoluysian atrophy (DRPLA), spinobulbar muscular atrophy (SBMA) and types of spinocerebellar ataxia (SCA). In these diseases, the pathogenic alleles usually contain 39 or more consecutive glutamine repeats. Higher repeat numbers lead to lower ages of onset. Patients with 40-60 glutamine repeats normally develop disease as adults, whereas patients with more than 60 repeats develop a juvenile onset disease. Each polyglutamine expansion disorder displays characteristic pathology, with neuronal loss evident in specific regions of the brain. It is believed that cells cannot properly dispose of proteins with overlong polyglutamine tracts, which over time leads to damage in nerve cells.
Host Mouse
Immunogen Recombinant protein
Subclass IgG1 kappa
Notes The epitope of the 1C2 antibody was found to be a homopolymeric glutamine stretch.The original Immunogen was the general transcription factor TATA box--binding protein (TBP) which contains a 38-glns stretch (Lescure &al). Other polyglutamine-containing proteins arerecognized by the 1C2 antibody, notably those involved in several human neurodegenrative diseasescaused by a CAG repeat expansion, like Huntington’s disease and spinocerebellar ataxia type 2,3 and7 (Trottier et al Nature). Importantly, for proteins involved in these neurodegenerative disorders, the1C2 antibody showed the remarkable property of detecting much better the pathological proteins thatcontain a polyglutamine expansion (>37 glns) than the wild type proteins (Trottier & al-1995,1998).1C2 has been used to identify new neurodegenerative disease caused by polyglutamine expansion andto help for cloning of the corresponding affected genes (Trottier 1995-1998 ; Imbert 1996; Stevanin1996).1C2 is also able to detect intracellular inclusions, which is a hallmark of such diseases (Paulson,1997).

Available in 100ul units as ascites
Research Area Neurobiology

References: 10 entries

Stevanin et al. 1996. Hum Mol Genet. 5(12):1887-92. PMID: 8968739.

Screening for proteins with polyglutamine expansions in autosomal dominant cerebellar ataxias.

Europe PMC ID: 8968739

Imbert et al. 1996. Nat Genet. 14(3):285-91. PMID: 8896557.

Mangiarini et al. 1996. Cell. 87(3):493-506. PMID: 8898202.

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice.

Europe PMC ID: 8898202

Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats.

Europe PMC ID: 8896557

Trottier et al. 1995. Nature. 378(6555):403-6. PMID: 7477379.

Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias.

Europe PMC ID: 7477379

Lescure et al. 1994. EMBO J. 13(5):1166-75. PMID: 7510635.

The N-terminal domain of the human TATA-binding protein plays a role in transcription from TATA-containing RNA polymerase II and III promoters.

Europe PMC ID: 7510635


Add a reference

References: 10 entries

Stevanin et al. 1996. Hum Mol Genet. 5(12):1887-92. PMID: 8968739.

Screening for proteins with polyglutamine expansions in autosomal dominant cerebellar ataxias.

Europe PMC ID: 8968739

Imbert et al. 1996. Nat Genet. 14(3):285-91. PMID: 8896557.

Mangiarini et al. 1996. Cell. 87(3):493-506. PMID: 8898202.

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice.

Europe PMC ID: 8898202

Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats.

Europe PMC ID: 8896557

Trottier et al. 1995. Nature. 378(6555):403-6. PMID: 7477379.

Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias.

Europe PMC ID: 7477379

Lescure et al. 1994. EMBO J. 13(5):1166-75. PMID: 7510635.

The N-terminal domain of the human TATA-binding protein plays a role in transcription from TATA-containing RNA polymerase II and III promoters.

Europe PMC ID: 7510635


Add a reference

References: 10 entries

Stevanin et al. 1996. Hum Mol Genet. 5(12):1887-92. PMID: 8968739.

Screening for proteins with polyglutamine expansions in autosomal dominant cerebellar ataxias.

Imbert et al. 1996. Nat Genet. 14(3):285-91. PMID: 8896557.

Mangiarini et al. 1996. Cell. 87(3):493-506. PMID: 8898202.

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice.

Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats.

Trottier et al. 1995. Nature. 378(6555):403-6. PMID: 7477379.

Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias.

Lescure et al. 1994. EMBO J. 13(5):1166-75. PMID: 7510635.

The N-terminal domain of the human TATA-binding protein plays a role in transcription from TATA-containing RNA polymerase II and III promoters.


Add a reference


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