|Positive WB detected in||Recombinant protein|
|Positive IHC detected in||human cerebellum tissue|
Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0
|Western Blot (WB)||WB : 1:500-1:2000|
|Immunohistochemistry (IHC)||IHC : 1:200-1:500|
|Sample-dependent, check data in validation data gallery|
24494-1-AP targets GP repeat in WB, IHC, IF, ELISA applications and shows reactivity with human samples.
|Host / Isotype||Rabbit / IgG|
|Full Name||GP 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 GP repeated sequence. The recombinant protein 6*His tag-SMARCA4 or GST tag-SMARCA4 which contains the sequence "GPGPGPGPG" were used to detect this antibody.
Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers.
Increased prevalence of granulovacuolar degeneration in C9orf72 mutation.
Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy.
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.
Hum Mol Genet
Modelling C9orf72 dipeptide repeat proteins of a physiologically relevant size.
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.
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Barbara (Verified Customer) (01-14-2019)