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

Anti C9orf72 Antibodies


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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. 

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

EELISA

EImmunohistochemistry

Antibody Characterization

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

References

1. DeJesus-Hernandez M, et al., Neuron 72: 245-256. 2011.
2. Renton AE, et al., Neuron 72: 257-268, 2011.
3. Mori K, et al., Science 339: 1335-1338, 2013.
4. Ash PEA, et al., Neuron 77: 639-646, 2013.
5. Mann DMA, et al., Acta Neuropathologica Communications 1: 68, 2013.
6. Konno T et al., Neuropathol Appl Neurobiol in press.

Product List

Product Name Cat# Quantity Price

Anti C9orf72 (Poly-GA), Human (Rabbit) Unlabeled DataSheet

CAC-TIP-C9-P01

50 UL ¥40,000
$534

Anti C9orf72 (Poly-GR), Human (Rabbit) Unlabeled DataSheet

CAC-TIP-C9-P02

50 UL ¥40,000
$534

Anti C9orf72 (Poly-GP), Human (Rabbit) Unlabeled DataSheet

CAC-TIP-C9-P03

50 UL ¥40,000
$534

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


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