|Sample-dependent, check data in validation data gallery|
24492-1-AP targets GA 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 "GA repeat" sequence. This antibody detects the GAGAGAGAGA peptide with dilution 1:32,000 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.
Altered MICOS Morphology and Mitochondrial Ion Homeostasis Contribute to Poly(GR) Toxicity Associated with C9-ALS/FTD.
Hum Mol Genet
Smcr8 deficiency disrupts axonal transport-dependent lysosomal function and promotes axonal swellings and gain of toxicity in C9ALS/FTD mouse models.
J Cell Sci
SFPQ regulates the accumulation of RNA foci and dipeptide repeat proteins from the expanded repeat mutation in C9orf72.
C9orf72 Hexanucleotide Expansions Are Associated with Altered Endoplasmic Reticulum Calcium Homeostasis and Stress Granule Formation in Induced Pluripotent Stem Cell-Derived Neurons from Patients with Amyotrophic Lateral Sclerosis and Frontotemporal Dementia.
Hum Mol Genet
Modelling C9orf72 dipeptide repeat proteins of a physiologically relevant size.