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Merck
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MABN2280

Sigma-Aldrich

Anti-FMR1polyG Antibody, clone 8FM-2F7

ascites fluid, clone 8FM-2F7, from mouse

Szinonimák:

Synaptic functional regulator FMR1, Fragile X mental retardation protein 1, FMRP, Protein FMR-1

Bejelentkezésa Szervezeti és Szerződéses árazás megtekintéséhez


About This Item

UNSPSC kód:
12352203
eCl@ss:
32160702
NACRES:
NA.41

biológiai forrás

mouse

antitest forma

ascites fluid

antitest terméktípus

primary antibodies

klón

8FM-2F7, monoclonal

faj reaktivitás

human

kiszerelés

antibody small pack of 25 μL

technika/technikák

immunofluorescence: suitable
immunohistochemistry: suitable (paraffin)
western blot: suitable

izotípus

IgG1κ

NCBI elérési szám

UniProt elérési szám

célzott transzláció utáni módosítás

unmodified

Géninformáció

human ... FMR1(2332)

Általános leírás

Synaptic functional regulator FMR1 (UniProt: Q06787; also known as Fragile X mental retardation protein 1, FMRP, Protein FMR-1) is encoded by the FMR1 gene (Gene ID: 2332) in human. FMR-1 is a multifunctional polyribosome-associated RNA-binding protein that plays a central role in neuronal development and synaptic plasticity through the regulation of alternative mRNA splicing, mRNA stability, mRNA dendritic transport and postsynaptic local protein synthesis of a subset of mRNAs. It plays a role in mRNA nuclear export and together with export factor NXF2, it is involved in the regulation of the NXF1 mRNA stability in neurons. It is also reported to stabilize the scaffolding postsynaptic density protein DLG4/PSD-95 and the myelin basic protein mRNAs in hippocampal neurons and glial cells, respectively and this stabilization is further increased in response to metabotropic glutamate receptor stimulation. A CGG repeat expansion (55-200) in the 5 UTR of FMR1 gene is reported to cause Fragile X-associated Tremor Ataxia Syndrome (FXTAS). The expanded CGG repeat is associated with elevated FMR1 mRNA expression that results and neurodegeneration. The CGG repeat expansion triggers repeat-associated non-ATG-initiated translation (RAN or RANT) within the 5 UTR of FMR1 mRNA, resulting in the production of fusion proteins (FMR1polyG) that contain N-terminal polyglycine fused to either C-terminal FMR1 in-frame sequence or one of two FMR1 frame-shift sequences. The FMR1polyG-positive aggregates closely resemble neuronal intranuclear inclusions seen in polyglutamine diseases and other protein-mediated neurodegenerative disorders. (Ref.: Buijsen, RAM., et al. (2014). Acta Neuropathol. Commun. 2:162; Todd, PK et al. (2013). Neuron. 78(3); 440-455).

Egyediség

Clone 8FM-2F7 specifically detects FMR1polyG in human cells.

Immunogén

Epitope: N-terminus
Ovalbumin-conjugated linear peptide with EAPLPGGVRQRGG sequence. Note: This protein is translated within the 5′UTR of the FMR1 gene and in a different frame resulting in a protein with different sequence and containing polyGlycine.

Alkalmazás

Anti-FMR1polyG, clone 8FM-2F7, Cat. No. MABN2280, is a highly specific mouse monoclonal antibody that targets Synaptic functional regulator FMR1 (FMR1polyG) and has been tested for use Immunofluorescence, Immunohistochemistry (Paraffin), and Western Blotting.
Research Category
Neuroscience
Western Blotting Analysis: A 1:500 dilution from a representative lot detected FMR1polyG in lysate from HEK293 cells overexpressing FMR1polyG.

Immunohistochemistry Analysis: A representative lot detected FMR1polyG in Immunohistochemistry applications (Buijsen, R.A., et. al. (2014). Acta Neuropathol Commun. 2:162; Sellier, C., et. al. (2017). Neuron. 93(2):331-347).

Western Blotting Analysis: A representative lot detected FMR1polyG in Western Blotting applications (Sellier, C., et. al. (2017). Neuron. 93(2):331-347).

Immunohistochemistry Analysis: A 1:50 dilution from a representative lot detected FMR1polyG in human cerebral cortex and human testis tissues.

Immunofluorescence Analysis: A representative lot detected FMR1polyG in Immunofluorescence applications (Buijsen, R.A., et. al. (2014). Acta Neuropathol Commun. 2:162; Sellier, C., et. al. (2017). Neuron. 93(2):331-347).

Minőség

Evaluated by Western Blotting in HEK293 cell lysates expressing FMR1polyG.

Western Blotting Analysis: A 1:500 dilution of this antibody detected FMR1polyG in HEK293 cell lysates expressing FMR1polyG.

Cél megnevezése

~35 kDa observed. Uncharacterized bands may be observed in some lysate(s).

Fizikai forma

Mouse monoclonal antibody in ascites without preservatives.
Unpurified

Tárolás és stabilitás

Stable for 1 year at -20°C from date of receipt. Handling Recommendations: Upon receipt and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.

Egyéb megjegyzések

Concentration: Please refer to lot specific datasheet.

Jogi nyilatkozat

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Dokumentumtár megtekintése

Xuan Phuoc Nguyen et al.
Genes, 13(3) (2022-03-26)
Fragile X-associated primary ovarian insufficiency (FXPOI) is characterized by oligo/amenorrhea and hypergonadotropic hypogonadism and is caused by the expansion of the CGG repeat in the 5'UTR of Fragile X Mental Retardation 1 (FMR1). Approximately 20% of women carrying an FMR1

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