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Antigenic Specificity | C9orf72 |
Clone | polyclonal |
Host Species | Chicken |
Reactive Species | human |
Isotype | n/a |
Format | IgY fraction |
Size | 100 µg |
Concentration | 1.03 mg/ml |
Applications | ELISA, Flow Cytometry, Immunohistochemistry, IF Microscopy |
Reviews / Ratings | If you have used this antibody, please help fellow researchers by submitting reviews to pAbmAbs and antYbuddY. |
Description | This affinity purified antibody is directed against human C9orf72. This product is an IgY fraction antibody purified from monospecific chicken egg yolks by a multi-step process which includes selective precipitation and salt fractionation followed by extensive dialysis against the buffer stated above. A BLAST analysis was used to suggest cross-reactivity with the antigen based on 100% homology with the immunizing sequence to mouse and rat. C9orf72 (Chromosome 9 Open Reading Frame 72) 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. It is a component of the C9orf72-SMCR8 complex, a complex that has guanine nucleotide exchange factor (GEF) activity and regulates autophagy. In the complex, C9orf72 and SMCR8 probably constitute the catalytic subunits that promote the exchange of GDP to GTP, converting inactive GDP-bound RAB8A and RAB39B into their active GTP-bound form, thereby promoting autophagosome maturation. The C9orf72-SMCR8 complex also acts as a regulator of autophagy initiation by interacting with the ATG1/ULK1 kinase complex and modulating its protein kinase activity. It positively regulates initiation of autophagy by regulating the RAB1A-dependent trafficking of the ATG1/ULK1 kinase complex to the phagophore which leads to autophagosome formation. And acts as a regulator of mTORC1 signaling by promoting phosphorylation of mTORC1 substrates. C9orf72 may be involved in regulating the maturation of phagosomes to lysosomes. It regulates actin dynamics in motor neurons by inhibiting the GTP-binding activity of ARF6, leading to ARF6 inactivation. This reduces the activity of the LIMK1 and LIMK2 kinases which are responsible for phosphorylation and inactivation of cofilin, leading to cofilin activation. It positively regulates axon extension and axon growth cone size in spinal motor neurons. C9orf72 plays a role within the hematopoietic system in restricting inflammation and the development of autoimmunity. 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. Diseases associated with C9orf72 include Frontotemporal Dementia And/Or Amyotrophic Lateral Sclerosis 1 and Huntington Disease-Like Syndrome Due To C9orf72 Expansions. Anti-C9orf72 Antibody is useful for researchers interested in Neuroscience Research. |
Immunogen | Anti-C9orf72 antibody was prepared from eggs of chickens laid after repeated immunizations with a synthetic peptide corresponding to a C-terminal portion of human C9orf72 surrounding aa 425-481 conjugated to Keyhole Limpet Hemocyanin (KLH). |
Other Names | Chicken Anti-Chromosome 9 Open Reading Frame 72 Antibody, Chicken Anti-C9orf72 Antibody, Guanine Nucleotide Exchange, Protein C9orf72, DENNL72, FTDALS1, ALSFTD, FTDALS |
Gene, Accession # | C9orf72, Gene ID: 203228, NCBI: NP_001242983.1, UniProt: Q96LT7 |
Catalog # | 200-901-MH2 |
Price | $475 |
Order / More Info | C9orf72 Antibody from ROCKLAND IMMUNOCHEMICALS INC. |
Product Specific References | n/a |