C9ORF72 antibody (Internal Region)

Catalog No. ABIN6260375

Product Details anti-C9ORF72 Antibody

Target: C9ORF72 (Chromosome 9 Open Reading Frame 72 (C9ORF72))

Binding Specificity: Internal Region

Reactivity: Human, Rat, Mouse

Host: Rabbit

Clonality: Polyclonal

Conjugate: This C9ORF72 antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (IHC)

Specificity: C9orf72 Antibody detects endogenous levels of total C9orf72.

Predicted Reactivity: Pig,Sheep,Rabbit

Purification: The antiserum was purified by peptide affinity chromatography using SulfoLink^TM Coupling Resin (Thermo Fisher Scientific).

Immunogen: A synthesized peptide derived from human C9orf72, corresponding to a region within the internal amino acids.

Isotype: IgG

Application Details

Application Notes: WB 1:500-1:2000, IHC 1:50-1:200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 mg/mL

Buffer: Rabbit IgG in phosphate buffered saline , pH 7.4, 150 mM NaCl, 0.02 % sodium azide and 50 % glycerol.

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20 °C. Stable for 12 months from date of receipt.

Expiry Date: 12 months

Target Details for C9ORF72

Target: C9ORF72 (Chromosome 9 Open Reading Frame 72 (C9ORF72))

Alternative Name: C9orf72 (C9ORF72 Products)

Synonyms: CZH9orf72 antibody, ALSFTD antibody, FTDALS antibody, AI840585 antibody, c9orf72 antibody, chromosome Z C9orf72 homolog antibody, chromosome 9 open reading frame 72 antibody, RIKEN cDNA 3110043O21 gene antibody, similar to RIKEN cDNA 3110043O21 antibody, chromosome 9 open reading frame 72 L homeolog antibody, CZH9orf72 antibody, C9orf72 antibody, 3110043O21Rik antibody, RGD1359108 antibody, c9orf72.L antibody

Background:

Description: 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).

Gene: C9orf72

Molecular Weight: 50-54 kDa,25-30 kDa

Gene ID: 203228

UniProt: Q96LT7