C9RANT Antibody

Pooled (GA)8, (GP)8 and (GR)8 peptides

Poly(GP)

This C9RANT antibody detects poly(GP) peptides produced via repeat associated non-ATG translation in central nervous system tissues from humans affected by an expanded hexanucleotide (GGGGCC) repeat in the C9ORF72 gene, the most common genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia (now termed c9FTD/ALS).

IgG

Polyclonal

Rabbit

Unpurified

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In ELISA, anti-C9RANT preferentially detects purified polyGP peptides, as well as polyGP peptides in human brain homogenates, by indirect immunoassay. For Western blot, the MW of endogenous polyGP peptides will depend on the size of the GGGGCC hexanucleotide repeat, as this will influence the length of the polyGP peptide synthesized. Insoluble polyGP peptides extracted from brain homogenates of humans affected by an expanded hexanucleotide (GGGGCC) repeat in the C9ORF72 gene may form high molecular weight smears and/or fail to enter the resolving gel. Anti-C9RANT preferentially detects GFP-tagged (GP)5, but not GFP-(GA)5 or GFP-(GR)5, in various cultured cell lines following transfection with respective expression vectors. In human tissue, polyGP peptides have been detected in cerebellar and frontal cortical tissues from c9FTD/ALS cases. In ICC/IF, anti-C9RANT detects exogenous polyGP peptides in cultured cell lines, and endogenous polyGP inclusions in tissues of the central nervous system from c9FTD/ALS cases. In humans, the staining is predominately cytoplasmic inclusions. In IHC-P, this antibody has been used with tissues of the central nervous system from humans harboring an expanded GGGGCC repeat in C9ORF72. Heat induced antibody retrieval is recommended.