Technical Data

520787
Clone Type
Polyclonal
Host
Rabbit
Source
Human
Isotype
IgG
Grade
Affinity Purified
Applications
E IHC WB
Crossreactivity
Hu Mo Rt
Shipping Temp
Blue Ice
Storage Temp
-20°C
Rabbit Anti-C9orf72
ALSFTD, C9orf72, chromosome 9 open reading frame 72, CI072_HUMAN, FTDALS, MGC23980, Protein C9orf72, RP11-27J8.2, Uncharacterized protein C9orf72|

Component of the C9orf72-SMCR8 complex, a complex that has guanine nucleotide exchange factor (GEF) activity and regulates autophagy (PubMed:27193190, PubMed:27103069, PubMed:27617292, PubMed:28195531). 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 (PubMed:27103069). 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 (PubMed:27617292). 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 (PubMed:27334615). Acts as a regulator of mTORC1 signaling by promoting phosphorylation of mTORC1 substrates (PubMed:27559131). Plays a role in endosomal trafficking (PubMed:24549040). May be involved in regulating the maturation of phagosomes to lysosomes (By similarity). Regulates actin dynamics in motor neurons by inhibiting the GTP-binding activity of ARF6, leading to ARF6 inactivation (PubMed:27723745). This reduces the activity of the LIMK1 and LIMK2 kinases which are responsible for phosphorylation and inactivation of cofilin, leading to cofilin activation (PubMed:27723745). Positively regulates axon extension and axon growth cone size in spinal motor neurons (PubMed:27723745). Plays a role within the hematopoietic system in restricting inflammation and the development of autoimmunity (By similarity).

Applications
Suitable for use in Western Blot, Immunohistochemistry and ELISA. Other applications not tested.
Recommended Dilution
Western Blot: 1:500-1:2000, incubate membrane with diluted antibody in TBS, 5% milk, 0.1% Tween20 at 4°C with gentle shaking, overnight. Immunohistochemistry: 1:50-1:200 ELISA (peptide): 1:20,000-1:40,000 Optimal dilutions to be determined by the researcher.
Storage and Stability
May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Immunogen
Synthetic peptide corresponding to human C9orf72. Species sequence homology: Porcine (100%), Sheep (100%), Rabbit (100%)
Form
Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
Purity
Purified by immunoaffinity chromatography.
Specificity
Recognizes endogenous levels of human C9orf72. Species Crossreactivity: mouse and rat

Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.

References
No references available
USBio References
No references available
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