C9orf72 Positive Control for STJ500332 peptide (STJ503805)

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STJ503805-5

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Applications: WB
Note: STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS.
Short Description: C9orf72 Positive Control for STJ500332 is synthetically produced from the sequence and is suitable for use in western blot applications.
Formulation: Provided as 100 uL ready-to-use, in SDS-PAGE sample buffer (Laemelli's buffer) containing Tris, pH 6.8, 1 % SDS, Glycerol and Bromophenolblue blue as tracking dye. The sample is reduced by adding 2% beta mercaptoethanol. The protein concentration is
Dilution Range: WB: 1:500
Storage Instruction: Store at-20°C for long term storage. Avoid freeze-thaw cycles.
Gene Symbol: C9orf72
Gene ID: 203228
Uniprot ID: CI072_HUMAN
Specificity: This is positive control is recommended for use in combination with C9orf72 antibody STJ500332.
Tissue Specificity Both isoforms are widely expressed, including kidney, lung, liver, heart, testis and several brain regions, such as cerebellum. Also expressed in the frontal cortex and in lymphoblasts (at protein level).
Function 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 ULK1/ATG1 kinase complex and modulating its protein kinase activity. As part of the C9orf72-SMCR8 complex, stimulates RAB8A and RAB11A GTPase activity in vitro. Positively regulates initiation of autophagy by regulating the RAB1A-dependent trafficking of the ULK1/ATG1 kinase complex to the phagophore which leads to autophagosome formation. Acts as a regulator of mTORC1 signaling by promoting phosphorylation of mTORC1 substrates. Plays a role in endosomal trafficking. May be involved in regulating the maturation of phagosomes to lysosomes. Promotes the lysosomal localization and lysosome-mediated degradation of CARM1 which leads to inhibition of starvation-induced lipid metabolism. 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 CFL1/cofilin activation. Positively regulates axon extension and axon growth cone size in spinal motor neurons. Required for SMCR8 protein expression and localization at pre- and post-synaptic compartments in the forebrain, also regulates protein abundance of RAB3A and GRIA1/GLUR1 in post-synaptic compartments in the forebrain and hippocampus. Plays a role within the hematopoietic system in restricting inflammation and the development of autoimmunity. Isoform 1: Regulates stress granule assembly in response to cellular stress. Isoform 2: Does not play a role in regulation of stress granule assembly in response to cellular stress.
Peptide Name Guanine Nucleotide Exchange Factor C9orf72
Cellular Localisation Nucleus
Cytoplasm
P-Body
Stress Granule
Endosome
Lysosome
Cytoplasmic Vesicle
Autophagosome
Secreted
Cell Projection
Axon
Growth Cone
Perikaryon
Detected In The Cytoplasm Of Neurons From Brain Tissue
Detected In The Nucleus In Fibroblasts
During Corticogenesis
Transitions From Being Predominantly Cytoplasmic To A More Even Nucleocytoplasmic Distribution
Isoform 1: Perikaryon
Dendrite
Presynapse
Postsynapse
Expressed Diffusely Throughout The Cytoplasm And Dendritic Processes Of Cerebellar Purkinje Cells
Also Expressed Diffusely Throughout The Cytoplasm Of Spinal Motor Neurons
Isoform 2: Nucleus Membrane
Peripheral Membrane Protein
Detected At The Nuclear Membrane Of Cerebellar Purkinje Cells And Spinal Motor Neurons
Also Shows Diffuse Nuclear Expression In Spinal Motor Neurons
Alternative Peptide Names Guanine Nucleotide Exchange Factor C9orf72 protein
C9orf72 protein
DENND9 protein
DENNL72 protein

Information sourced from Uniprot.org

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