WH0002332M1
Monoclonal Anti-FMR1 antibody produced in mouse
clone 2D4, purified immunoglobulin, buffered aqueous solution
Synonim(y):
Anti-FMRP, Anti-FRAXA, Anti-MGC87458, Anti-fragile X mental retardation 1
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About This Item
Polecane produkty
pochodzenie biologiczne
mouse
białko sprzężone
unconjugated
forma przeciwciała
purified immunoglobulin
rodzaj przeciwciała
primary antibodies
klon
2D4, monoclonal
Postać
buffered aqueous solution
reaktywność gatunkowa
human
metody
indirect ELISA: suitable
western blot: 1-5 μg/mL
izotyp
IgG1κ
numer dostępu GenBank
numer dostępu UniProt
Warunki transportu
dry ice
temp. przechowywania
−20°C
docelowa modyfikacja potranslacyjna
unmodified
informacje o genach
human ... FMR1(2332)
Opis ogólny
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)
Immunogen
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
Działania biochem./fizjol.
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.
Postać fizyczna
Solution in phosphate buffered saline, pH 7.4
Informacje prawne
GenBank is a registered trademark of United States Department of Health and Human Services
Oświadczenie o zrzeczeniu się odpowiedzialności
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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polecane
Kod klasy składowania
10 - Combustible liquids
Temperatura zapłonu (°F)
Not applicable
Temperatura zapłonu (°C)
Not applicable
Środki ochrony indywidualnej
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
Certyfikaty analizy (CoA)
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Masz już ten produkt?
Dokumenty związane z niedawno zakupionymi produktami zostały zamieszczone w Bibliotece dokumentów.
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
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