Anti-C9ORF72 Antibody Picoband®

SKU: A00419-1

Size: 100 μg

Storage: Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: Flow Cytometry,IHC-P,WB

Application Details: Western blot, 0.25-0.5μg/ml, Human, Mouse, Rat
Flow Cytometry (Fixed), 1-3μg/1x10⁶ cells, Human

Gene Name: C9ORF72

Specificity: No cross reactivity with other proteins.

Background: C9orf72 (chromosome 9 open reading frame 72) is a protein which in humans is encoded by the gene C9orf72. It is mapped to 9p21.2. The protein encoded by this gene plays an important role in the regulation of endosomal trafficking, and has been shown to interact with Rab proteins that are involved in autophagy and endocytic transport. Expansion of a GGGGCC repeat from 2-22 copies to 700-1600 copies in the intronic sequence between alternate 5' exons in transcripts from this gene is associated with 9p-linked ALS (amyotrophic lateral sclerosis) and FTD (frontotemporal dementia) (PMID: 21944778, 21944779). Studies suggest that hexanucleotide expansions could result in the selective stabilization of repeat-containing pre-mRNA, and the accumulation of insoluble dipeptide repeat protein aggregates that could be pathogenic in FTD-ALS patients (PMID: 23393093). Alternative splicing results in multiple transcript variants encoding different isoforms.

Immunogen: A synthetic peptide corresponding to a sequence at the N-terminus of human C9ORF72, which shares 96.4% amino acid (aa) sequence identity with both mouse and rat C9ORF72.

Clonality: Polyclonal

Contents: Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.

Purification: Immunogen affinity purified.

Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Reference: 1. DeJesus-Hernandez M, Mackenzie IR, Boeve BF, Boxer AL, Baker M, Rutherford NJ, Nicholson AM, Finch NA, Flynn H, Adamson J, Kouri N, Wojtas A, Sengdy P, Hsiung GY, Karydas A, Seeley WW, Josephs KA, Coppola G, Geschwind DH, Wszolek ZK, Feldman H, Knopman DS, Petersen RC, Miller BL, Dickson DW, Boylan KB, Graff-Radford NR, Rademakers R (2011). Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron. 72 (2): 245–56. 2. Babić Leko, M; Župunski, V; Kirincich, J; Smilović, D; Hortobágyi, T; Hof, PR; Šimić, G (2019). Molecular Mechanisms of Neurodegeneration Related to C9orf72 Hexanucleotide Repeat Expansion. Behavioural neurology. 2019: 2909168. doi:10.1155/2019/2909168. 3. Bigio EH (2011). C9ORF72, the new gene on the block, causes C9FTD/ALS: new insights provided by neuropathology. Acta Neuropathol. 122 (6): 653–5. doi:10.1007/s00401-011-0919-7.

Uniprot ID: Q96LT7

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate: This is the unconjugated antibody. It is also available with 10 conjugation options, including Biotin, Cy3, Fluoro488, Fluoro550, Fluoro594, FITC, HRP, APC, PE, and Fluoro647. A carrier-free (BSA-free) version is also available. Additional charges may apply for some options. Please contact us for a quote

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 65 kDa

Calculated Molecular Weight: 14917 MW

Gene ID: 203228

Protein Name: Guanine nucleotide exchange factor C9orf72

Gene Full Name: C9orf72-SMCR8 complex subunit

Synonyms: C9orf72, C9RANT, RP11 27J8.2