|Positive WB detected in||Recombinant protein|
|Western Blot (WB)||WB : 1:500-1:2000|
|Sample-dependent, check data in validation data gallery|
23978-1-AP targets GR 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 GR repeated sequence. The recombinant proteins GST-SNRPD1 and 6*His tag- SNRPD1 which contains the sequence “GRGRGRGRGRGRGRGRGR” were used to detect this antibody. Both proteins can be recognized by the antibody 23978-1-AP.
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.
Acta Neuropathol Commun
Co-expression of C9orf72 related dipeptide-repeats over 1000 repeat units reveals age- and combination-specific phenotypic profiles in Drosophila.
Hum Mol Genet
Smcr8 deficiency disrupts axonal transport-dependent lysosomal function and promotes axonal swellings and gain of toxicity in C9ALS/FTD mouse models.
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.