Mouse Anti-C9orf72 Monoclonal Antibody (CBP2699)

Product Overview

Description

The C9ORF72-hexanucleotide repeat expansions have been recently identified as genetic markers in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). FTLD-TDP has five subtypes: Sporadic FTLD, GRN mutation FTLD, TARDBP mutation FTLD, VCP mutation FTLD and C9ORF72 mutation FTLD. The C9ORF72 repeat expansions may indicate a worse prognosis in ALS.

Expression Host

Mammalian cells

Immunogen

C9orf72 fusion protein Ag21080

Species Reactivity

Human; Mouse; Rat

Clonality

Monoclonal

Host Species

Mouse

Isotype

IgG2a

Clone Number

CBP2699

Applications

IF; IHC; IP; WB; ELISA

Application Notes

IF : 1:20-1:200; IHC : 1:20-1:200; IP : 0.5-4.0 ug; WB : 1:500-1:1000

Research Areas

Neurotransmission

Conjugation

Unconjugated

Product Properties

Form

Liquid

Formulation

PBS, 0.1% sodium azide and 50% glycerol pH 7.3.

Preservatives

Yes

Concentration

1173 μg/ml

Purification

Antigen affinity purification

Purity

>95%, as determined by SDS-PAGE

Shipping

The product is shipped at 4°C. Upon receipt, store it immediately at the temperature recommended below.

Storage

Store at -20°C. Stable for one year after shipment.

Research Use Only

For research use only, not for diagnostic or therapeutic use.

Target

C9orf72

Official Name

C9orf72

Full Name

Chromosome 9 open reading frame 72

Alternative Names

ALSFTD;
C9orf72;
chromosome 9 open reading frame 72;
CI072_HUMAN;
FTDALS;
MGC23980;
Protein C9orf72;
RP11-27J8.2;

Gene ID

203228(Human); 73205(Mouse); 313155(Rat)

Uniprot ID

Q96LT7(Human); Q6DFW0(Mouse); Q66HC3(Rat)

References

1. Sullivan, P. M., Zhou, X., Robins, A. M., Paushter, D. H., Kim, D., Smolka, M. B., & Hu, F. (2016). The ALS/FTLD associated protein C9orf72 associates with SMCR8 and WDR41 to regulate the autophagy-lysosome pathway. Acta neuropathologica communications, 4(1), 51. 2. Saberi, S., Stauffer, J. E., Jiang, J., Garcia, S. D., Taylor, A. E., Schulte, D.,... & Rodriguez, M. J. (2018). Sense-encoded poly-GR dipeptide repeat proteins correlate to neurodegeneration and uniquely co-localize with TDP-43 in dendrites of repeat-expanded C9orf72 amyotrophic lateral sclerosis. Acta neuropathologica, 135(3), 459-474. 3. Leskelä, S., Huber, N., Rostalski, H., Natunen, T., Remes, A. M., Takalo, M.,... & Haapasalo, A. (2019). C9orf72 Proteins Regulate Autophagy and Undergo Autophagosomal or Proteasomal Degradation in a Cell Type-Dependent Manner. Cells, 8(10), 1233.

Publications