|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.
|Cited Reactivity||human, mouse, Drosophila, zebrafish|
|Host / Isotype||Rabbit / IgG|
|Full Name||GR repeat|
|Gene ID (NCBI)|
|Purification Method||Antigen affinity purification|
|Storage Buffer||PBS with 0.02% sodium azide and 50% glycerol pH 7.3.|
|Storage Conditions||Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage.|
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.
The C9orf72 repeat expansion disrupts nucleocytoplasmic transport.
C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins.
Stress Granule Assembly Disrupts Nucleocytoplasmic Transport.
RPS25 is required for efficient RAN translation of C9orf72 and other neurodegenerative disease-associated nucleotide repeats.
A zebrafish model for C9orf72 ALS reveals RNA toxicity as a pathogenic mechanism.
Increased prevalence of granulovacuolar degeneration in C9orf72 mutation.