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Mouse Anti-GNAO1 Recombinant Antibody (CBLG1-1410) (CBMAB-G4112-LY)

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Datasheet
SDS
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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal
Mouse
Clone
CBLG1-1410
Application
WB
Specificity
Mouse
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Concentration
1 mg/mL
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.
More Infomation

Target

Full Name
guanine nucleotide binding protein, alpha O
Introduction
The protein encoded by this gene represents the alpha subunit of the Go heterotrimeric G-protein signal-transducing complex. Defects in this gene are a cause of early-onset epileptic encephalopathy. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Aug 2015]
Entrez Gene ID
14681
UniProt ID
P18872
Alternative Names
AW050213; Galphao; Gnao; alphaO
Function
Guanine nucleotide-binding proteins (G proteins) are involved as modulators or transducers in various transmembrane signaling systems. The G(o) protein function is not clear. Stimulated by RGS14.
Biological Process
Adenylate cyclase-modulating G protein-coupled receptor signaling pathway Source: GO_Central
Aging Source: Ensembl
Dopamine receptor signaling pathway Source: GO_Central
Forebrain development Source: Ensembl
Locomotory behavior Source: Ensembl
Muscle contraction Source: ProtInc
Negative regulation of calcium ion transport Source: Ensembl
Neuron projection development Source: Ensembl
Odontogenesis of dentin-containing tooth Source: Ensembl
Positive regulation of GTPase activity Source: Ensembl
Regulation of heart contraction Source: Ensembl
Response to cytokine Source: Ensembl
Response to drug Source: Ensembl
Response to hydrogen peroxide Source: Ensembl
Response to morphine Source: Ensembl
Cellular Location
Cell membrane; Membrane
Involvement in disease
Developmental and epileptic encephalopathy 17 (DEE17):
A severe neurologic disorder characterized by onset of intractable seizures in the first weeks or months of life and usually associated with EEG abnormalities. Affected infants have very poor psychomotor development and may have brain abnormalities, such as cerebral atrophy or thin corpus callosum. Some patients may show involuntary movements.
Neurodevelopmental disorder with involuntary movements (NEDIM):
A neurodevelopmental disorder manifesting with a wide range of clinical symptoms. Clinical features range from severe motor and cognitive impairment with marked choreoathetosis, self-injurious behavior and epileptic encephalopathy, to a milder phenotype featuring moderate developmental delay associated with complex stereotypies, mainly facial dyskinesia, and mild epilepsy. Hyperkinetic movements are often exacerbated by specific triggers, such as illness, emotion and high ambient temperature. Some patients have brain abnormalities, such as cerebral atrophy or thin corpus callosum.
PTM
Histaminylated at Gln-205 residues by TGM2.

Katanaev, V. L., Valnohova, J., Silachev, D. N., Larasati, Y. A., & Koval, A. (2023). Pediatric GNAO1 encephalopathies: from molecular etiology of the disease to drug discovery. Neural Regeneration Research, 18(10), 2188-2189.

Wirth, T., Garone, G., Kurian, M. A., Piton, A., Millan, F., Telegrafi, A., ... & Anheim, M. (2022). Highlighting the dystonic phenotype related to GNAO1. Movement Disorders, 37(7), 1547-1554.

Wang, D., Dao, M., Muntean, B. S., Giles, A. C., Martemyanov, K. A., & Grill, B. (2022). Genetic modeling of GNAO1 disorder delineates mechanisms of Gαo dysfunction. Human Molecular Genetics, 31(4), 510-522.

Di Rocco, M., Galosi, S., Lanza, E., Tosato, F., Caprini, D., Folli, V., ... & Martinelli, S. (2022). Caenorhabditis elegans provides an efficient drug screening platform for GNAO1-related disorders and highlights the potential role of caffeine in controlling dyskinesia. Human Molecular Genetics, 31(6), 929-941.

Axeen, E., Bell, E., Viehoever, A. R., Schreiber, J. M., Sidiropoulos, C., & Goodkin, H. P. (2021). Results of the first GNAO1-related neurodevelopmental disorders caregiver survey. Pediatric Neurology, 121, 28-32.

Kelly, M., Park, M., Mihalek, I., Rochtus, A., Gramm, M., Pérez‐Palma, E., ... & Murdock, D. R. (2019). Spectrum of neurodevelopmental disease associated with the GNAO1 guanosine triphosphate–binding region. Epilepsia, 60(3), 406-418.

Schirinzi, T., Garone, G., Travaglini, L., Vasco, G., Galosi, S., Rios, L., ... & Leuzzi, V. (2019). Phenomenology and clinical course of movement disorder in GNAO1 variants: results from an analytical review. Parkinsonism & Related Disorders, 61, 19-25.

Koy, A., Cirak, S., Gonzalez, V., Becker, K., Roujeau, T., Milesi, C., ... & Cif, L. (2018). Deep brain stimulation is effective in pediatric patients with GNAO1 associated severe hyperkinesia. Journal of the Neurological Sciences, 391, 31-39.

Feng, H., Khalil, S., Neubig, R. R., & Sidiropoulos, C. (2018). A mechanistic review on GNAO1-associated movement disorder. Neurobiology of disease, 116, 131-141.

Waak, M., Mohammad, S. S., Coman, D., Sinclair, K., Copeland, L., Silburn, P., ... & Malone, S. (2018). GNAO1-related movement disorder with life-threatening exacerbations: movement phenomenology and response to DBS. Journal of Neurology, Neurosurgery & Psychiatry, 89(2), 221-222.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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