Anti-C9ORF72 (Poly-GA) pAb — prepared from rabbits immunized with poly (GA)8 — recognizes poly (GA) dipeptide repeat proteins.
Validated for ELISA and IHC(p), this antibody is useful for immunohistochemical and biochemical studies of C9ORF72 (Poly-GA) dipeptide species in diseased brains.
Background
Anti-C9ORF72 (Poly-GA) pAb — prepared from rabbits immunized with poly (GA)8 — recognizes poly (GA) dipeptide repeat proteins. Validated for ELISA and IHC(p), this antibody is useful for immunohistochemical and biochemical studies of C9ORF72 (Poly-GA) dipeptide species in diseased brains.
In 2011 hexanucleotide expansions in the C9ORF72 gene were identified in patients with frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). GGGGCC expansions are characterized pathologically by the presence of TDP-43 negative and p62 positive inclusions in granule cells of the cerebellum and in cells of the 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.
Specifications
Product type primary antibody
Immunogen poly (GA)8
Raised in rabbit
Source anti-serum
Form liquid anti-serum with 0.1% NaN3 as a preservative
Volume 50 uL
Label unlabeled
Specificity poly (GA)8
Cross reactivity human
Storage below -20°C. (below -70°C for prolonged storage). Aliquot to avoid cycles of freeze/thaw.
Recommended dilutions
ELISA 1/500-1/2000
Immunohistochemistry 1/500-1/2000
Other applications have not been tested
Optimal dilutions/ concentrations should be determined by the end user.
References
David MA Mann, et al. Dipeptide repeat proteins are present in the p62 positive inclusions in patients with frontotemporal lobar degeneration and motor neuron disease associated with expansions in C9ORF72. Acta Neuropathologica Communications (2013) 1:68. PMID 24252525
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 Neuron Disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins. Neuropathol Appl Neurobiol. 2015 Nov 5. doi: 10.1111/nan.12292. 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. doi: 10.1111/nan.12178. Epub 2015 Apr 30. 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. doi: 10.1186/2051-5960-2-70. PMID: 24950788
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. doi: 10.1111/nan.12157. No abstract available. PMID: 24861677
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