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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: 5 entries

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

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

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

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

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


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References: 5 entries

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

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

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

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

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


Add a reference

References: 5 entries

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

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

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

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

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


Add a reference


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