WH0002332M1
Monoclonal Anti-FMR1 antibody produced in mouse
clone 2D4, purified immunoglobulin, buffered aqueous solution
Sinônimo(s):
Anti-FMRP, Anti-FRAXA, Anti-MGC87458, Anti-fragile X mental retardation 1
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About This Item
Produtos recomendados
fonte biológica
mouse
Nível de qualidade
conjugado
unconjugated
forma do anticorpo
purified immunoglobulin
tipo de produto de anticorpo
primary antibodies
clone
2D4, monoclonal
Formulário
buffered aqueous solution
reatividade de espécies
human
técnica(s)
indirect ELISA: suitable
western blot: 1-5 μg/mL
Isotipo
IgG1κ
nº de adesão GenBank
nº de adesão UniProt
Condições de expedição
dry ice
temperatura de armazenamento
−20°C
modificação pós-traducional do alvo
unmodified
Informações sobre genes
human ... FMR1(2332)
Descrição geral
The protein encoded by this gene binds RNA and is associated with polysomes. The encoded protein may be involved in mRNA trafficking from the nucleus to the cytoplasm. A trinucleotide repeat (CGG) in the 5′ UTR is normally found at 6-53 copies, but an expansion to 55-230 repeats is the cause of fragile X syndrome. Expansion of the trinucleotide repeat may also cause one form of premature ovarian failure (POF1). (provided by RefSeq)
Imunogênio
FMR1 (NP_002015, 121 a.a. ~ 220 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
ATKDTFHKIKLDVPEDLRQMCAKEAAHKDFKKAVGAFSVTYDPENYQLVILSINEVTSKRAHMLIDMHFRSLRTKLSLIMRNEEASKQLESSRQLASRFH
Sequence
ATKDTFHKIKLDVPEDLRQMCAKEAAHKDFKKAVGAFSVTYDPENYQLVILSINEVTSKRAHMLIDMHFRSLRTKLSLIMRNEEASKQLESSRQLASRFH
Ações bioquímicas/fisiológicas
Fragile X mental retardation 1 (FMR1) plays a vital role in neuronal development and maturation. Deletion or lack of expression of the gene encoding FMRP results in fragile X syndrome. FMRP1 acts as a vital element of messenger ribonucleoprotein complexes present within the translation apparatus, and is involved in the regulation of mRNA translation.
forma física
Solution in phosphate buffered saline, pH 7.4
Informações legais
GenBank is a registered trademark of United States Department of Health and Human Services
Exoneração de responsabilidade
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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Código de classe de armazenamento
10 - Combustible liquids
Ponto de fulgor (°F)
Not applicable
Ponto de fulgor (°C)
Not applicable
Equipamento de proteção individual
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
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Alain Y Dury et al.
PLoS genetics, 9(10), e1003890-e1003890 (2013-11-10)
Fragile X syndrome is caused by loss of function of a single gene encoding the Fragile X Mental Retardation Protein (FMRP). This RNA-binding protein, widely expressed in mammalian tissues, is particularly abundant in neurons and is a component of messenger
Damien Sanlaville et al.
Orphanet journal of rare diseases, 4, 4-4 (2009-02-24)
Distal Xq duplications refer to chromosomal disorders resulting from involvement of the long arm of the X chromosome (Xq). Clinical manifestations widely vary depending on the gender of the patient and on the gene content of the duplicated segment. Prevalence
Laetitia Davidovic et al.
Human molecular genetics, 15(9), 1525-1538 (2006-03-31)
The fragile X syndrome, the leading cause of inherited mental retardation, is due to the inactivation of the fragile mental retardation 1 gene (FMR1) and the subsequent absence of its gene product FMRP. This RNA-binding protein is thought to control
D Devys et al.
Nature genetics, 4(4), 335-340 (1993-08-01)
Fragile X mental retardation syndrome is caused by the unstable expansion of a CGG repeat in the FMR-1 gene. In patients with a full mutation, abnormal methylation results in suppression of FMR-1 transcription. FMR-1 is expressed in many tissues but
Jocelyn N Galloway et al.
Human molecular genetics, 23(22), 5906-5915 (2014-07-06)
Determining the molecular mechanism(s) leading to Purkinje neuron loss in the neurodegenerative disorder fragile X-associated tremor/ataxia syndrome (FXTAS) is limited by the complex morphology of this cell type. Purkinje neurons are notoriously difficult to isolate and maintain in culture presenting
Global Trade Item Number
| SKU | GTIN |
|---|---|
| WH0002332M1-100UG | 4061831632941 |
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