WB
H M R
Endogenous
49
Rabbit
#Q96LT7
203228
Product Information
Product Usage Information
| Application | Dilution |
|---|---|
| Western Blotting | 1:1000 |
Storage
Specificity / Sensitivity
Species Reactivity:
Human, Mouse, Rat
Source / Purification
Polyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Ala202 of human C9orf72 protein. Antibodies are purified by protein A and peptide affinity chromatography.
Background
The expansion of hexanucleotide GGGGCC repeats in the C9orf72 gene causes chromosome 9p-linked neurodegenerative diseases amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) (1,2). The specific mechanism by which of these repeats contributes to disease etiology is currently an active area of investigation (3). Several gain of function mechanisms have been proposed. These mechanisms include toxicity from C9orf72 RNA containing the hexanucleotide repeats (4) and toxicity generated from dipeptide repeat proteins produced by repeat-associated non-ATG translation (5). In addition to gain of function mechanisms, the genetic hexanucleotide repeat expansions may cause a loss of function of the C9orf72 protein. C9orf72 contains a predicted DENN (differentially expressed in normal and neoplastic cells) domain that typically functions as guanine exchange factors for Rab GTPases, proteins that play key regulatory roles in membrane trafficking (6). Consistent with C9orf72 normally functioning in membrane trafficking, biochemical and genetic studies revealed that C9orf72 forms a protein complex with Sim-Magenis chromosome region 8 (SMCR8) and WD repeat-containing protein 41 (WDR41) to regulate the autophagy-lysosomal pathway (7), suggesting that C9orf72-dependent alterations in the autophagy-lysosomal pathway might contribute to ALS/FTD pathology.
- DeJesus-Hernandez, M. et al. (2011) Neuron 72, 245-56.
- Renton, A.E. et al. (2011) Neuron 72, 257-68.
- Balendra, R. and Isaacs, A.M. (2018) Nat Rev Neurol 14, 544-58.
- Zhang, Y.J. et al. (2019) Science 363, pii: eaav2606. doi: 10.1126/science.aav2606.
- Cleary, J.D. et al. (2018) J Biol Chem 293, 16127-41.
- Amick, J. and Ferguson, S.M. (2017) Traffic 18, 267-76.
- Sullivan, P.M. et al. (2016) Acta Neuropathol Commun 4, 51.
Species Reactivity
Species reactivity is determined by testing in at least one approved application (e.g., western blot).
Western Blot Buffer
IMPORTANT: For western blots, incubate membrane with diluted primary antibody in 5% w/v BSA, 1X TBS, 0.1% Tween® 20 at 4°C with gentle shaking, overnight.
Applications Key
WB: Western Blotting
Cross-Reactivity Key
H: human M: mouse R: rat Hm: hamster Mk: monkey Vir: virus Mi: mink C: chicken Dm: D. melanogaster X: Xenopus Z: zebrafish B: bovine Dg: dog Pg: pig Sc: S. cerevisiae Ce: C. elegans Hr: horse GP: Guinea Pig Rab: rabbit All: all species expected
Trademarks and Patents
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