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For Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Lobar Degeneration (FTLD) research

Anti C9orf72 Antibodies

Go to Antibodies for Neurodegenerative Disease Research page

Background

Hexanucleotide expansions in C9orf72 gene was identified in patients with frontotemporal lobar degeneration (FTLD) and Amyotrophic Lateral Sclerosis (ALS) in 2011. GGGGCC expansions are characterised pathologically by the presence of TDP-43 negative and p62 positive inclusions in granule cells of cerebellum and in cells of dentate gyrus and CA4 area of the hippocampus. It was reported that these inclusions included dipeptide repeat proteins, poly-GA, poly-GR and poly GP, arising from a putative non-ATG initiated sense translation of the GGGGCC expansion. These antibodies are powerful tools for IHC analysis of neurodegenerative diseases. 

Application

・ELISA

・Immunohistochemistry

Antibody Characterization

Immunohistochemistry of C9orf72 detection with Poly GA antibody in cerebellum granular cell layer

Go to Antibodies for Neurodegenerative Disease Research page

Product List

Product Name Cat# Quantity Price

Anti C9orf72 (Poly-GA)

CAC-TIP-C9-P01 50UL

¥ 40,000
$ 534
€ 400

Anti C9orf72 (Poly-GR)

CAC-TIP-C9-P02 50UL

¥ 40,000
$ 534
€ 400

Anti C9orf72 (Poly-GP)

CAC-TIP-C9-P03 50UL

¥ 40,000
$ 534
€ 400

References
  • DeJesus-Hernandez M, et al., Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron 72: 245-256. 2011. PMID: 21944778
  • Renton AE, et al., A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron 72: 257-268, 2011. PMID: 21944779
  • Mori K, et al., The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS. Science 339: 1335-1338, 2013. PMID: 23393093
  • Ash PEA, et al., Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS. Neuron 77: 639-646, 2013. PMID: 23415312
  • Mann DMA, et al., Dipeptide repeat proteins are present in the p62 positive inclusions in patients with frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72. Acta Neuropathol Commun. 2013 Oct 14;1:68. PMID: 24252525
  • Konno T, et al. C9ORF72 repeat-associated non-ATG-translated polypeptides are distributed independently of TDP-43 in a Japanese patient with c9ALS. Neuropathol Appl Neurobiol. 2014 Oct;40(6):783-8. PMID: 24861677
  • Tan RH, et al. Cerebellar neuronal loss in als cases with ATXN2 intermediate repeat expansions. Ann Neurol. 2015 Nov 24. doi: 10.1002/ana.24565. PMID:26599997
  • Davidson Y, et al. Neurodegeneration in Frontotemporal Lobar Degeneration and Motor Neurone Disease associated with expansions in C9orf72is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins. Neuropathol Appl Neurobiol. 2015 Nov 5. PMID: 26538301
  • Baborie A, et al. Accumulation of dipeptide repeat proteins predates that of TDP-43 in frontotemporal lobar degeneration associated with hexanucleotide repeat expansions in C9ORF72 gene. Neuropathol Appl Neurobiol. 2015 Aug;41(5):601-12. PMID: 25185840
  • Davidson YS, et al. Brain distribution of dipeptide repeat proteins in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72. Acta Neuropathol Commun. 2014 Jun 20;2:70. PMID: 24950788

To be used for research only. DO NOT use for human gene therapy or clinical diagnosis.