|Sample-dependent, check data in validation data gallery|
23979-1-AP targets PR repeat in WB, IHC, IF, Dot Blot,ELISA applications and shows reactivity with human samples.
The C9orf72 "GGGGCC" repeat sequence codes five repeat peptide "GA repeat; GAGAGAGAGA", "GP repeat; GPGPGPGPG", "GR repeat; GRGRGRGRG", "AP repeat; APAPAPAPA" and "PR repeat; PRPRPRPRP". It was described previously that aggregated forms of poly-GA and poly-GP proteins do not enter the separation gel (PMID: 26374446).This antibody is used to detect the "PR repeat" sequence. This antibody detects the PRPRPRPRP peptide with dilution 1:4,500 in Elisa.
Neuropathol Appl Neurobiol
Neurodegeneration in Frontotemporal Lobar Degeneration and Motor Neurone Disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins.
Identification and therapeutic rescue of autophagosome and glutamate receptor defects in C9ORF72 and sporadic ALS neurons.
Altered MICOS Morphology and Mitochondrial Ion Homeostasis Contribute to Poly(GR) Toxicity Associated with C9-ALS/FTD.
C9orf72-Associated Arginine-Rich Dipeptide Repeat Proteins Reduce the Number of Golgi Outposts and Dendritic Branches in Drosophila Neurons.
Biochim Biophys Acta Mol Cell Res
Expression of C9orf72 hexanucleotide repeat expansion leads to formation of RNA foci and dipeptide repeat proteins but does not influence autophagy or proteasomal function in neuronal cells.
J Cell Sci
SFPQ regulates the accumulation of RNA foci and dipeptide repeat proteins from the expanded repeat mutation in C9orf72.