|Positive ELISA detected in||peptide|
|Enzyme-linked Immunosorbent Assay (ELISA)||ELISA : 1:10-1:100|
|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.
|Cited Reactivity||human, mouse, rat, Drosophila|
|Host / Isotype||Rabbit / IgG|
|Full Name||PR 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. 20ul sizes contain 0.1% BSA.|
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.
RPS25 is required for efficient RAN translation of C9orf72 and other neurodegenerative disease-associated nucleotide repeats.
Antisense Proline-Arginine RAN Dipeptides Linked to C9ORF72-ALS/FTD Form Toxic Nuclear Aggregates that Initiate In Vitro and In Vivo Neuronal Death.
Motor dysfunction and neurodegeneration in a C9orf72 mouse line expressing poly-PR.
Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers.
NDST3 deacetylates α-tubulin and suppresses V-ATPase assembly and lysosomal acidification.
The RNA exosome complex degrades expanded hexanucleotide repeat RNA in C9orf72 FTLD/ALS.