U-Blot® FMR1 Rabbit mAb

U-Blot® FMR1 Rabbit mAb
U-Blot® FMR1 Rabbit mAb
$268.00
Size:50μL
SKU: WR4994-50

Catalog No.: WR4994

Specification: 50μL/100μL

Stock: In stock

U-Blot® FMR1 Rabbit mAb Learn more

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Product information

Product detailsSpecificationsQ & AEducation

Background：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). Multiple alternatively spliced transcript variants that encode different protein isoforms and which are located in different cellular locations have been described for this gene. [provided by RefSeq, May 2010],

Product Overview

Catalog No.
WR4994
SKU
WR4994-50
Category
Rabbit mAbs
Product Type
Other
Size
50μL/100μL
Stock Status
In stock
Available Stock
20
Minimum Order Quantity
1

Storage & Compliance

Storage
-15°C to -25°C/1 year(Do not lower than -25°C)
Research Use Only
Yes

Additional Specifications

Target
FMR1
Reactivity
Human,Mouse,Rat
Application
WB,IHC,IF,ELISA
MW(Calculated)
71kD
MW(Observed)
70-77kD
Host Species
Rabbit
Isotype
IgG,Kappa
Conjugate/Modification
Unmodified
Modification site
--
Recommended Dilution Ratio
IHC 1:200-1:1000;WB 1:2000-1:10000;IF 1:200-1:1000;ELISA 1:5000-1:20000;
Form
PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
Source
--
Purification
Protein A
Purity
--
storageCondition
-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
--
Clonality
Monoclonal
Clone No.
PT1226R
Immunogen
--
Sequence
--
Specificity
Endogenous
Gene Name
FMR1
Protein Name
Fragile X mental retardation 1 protein
Other Name
FMR1;Fragile X mental retardation protein 1;FMRP;Protein FMR-1
Organism-1
Human
Gene ID-1
2332
SwissProt-1
Q06787
Organism-2
Mouse
Gene ID-2
--
SwissProt-2
P35922
Organism-3
--
Gene ID-3
--
SwissProt-3
--
Organism-4
--
gene ID-4
--
SwissProt-4
--
Cellular Localization
Alternative products:At least 12 different isoforms are produced,Disease:Defects in FMR1 are the cause of fragile X syndrome. [MIM:300624]. It is a common genetic disease (has a prevalence of one in every 2000 children) which is characterized by moderate to severe mental retardation, macroorchidism (enlargement of the testicles), large ears, prominent jaw, and high-pitched, jocular speech. The defect in most fragile X syndrome patients results from an amplification of a CGG repeat region which is directly in front of the coding region.,Disease:Defects in FMR1 are the cause of fragile X tremor/ataxia syndrome (FXTAS) [MIM:300623]. In FXTAS, the expanded repeats range in size from 55 to 200 repeats and are referred to as 'premutations'. Full repeat expansions with greater than 200 repeats results in fragile X mental retardation syndrome [MIM:300624]. Carriers of the premutation typically do not show the full fragile X syndrome phenotype, but comprise a subgroup that may have some physical features of fragile X syndrome or mild cognitive and emotional problems.,Function:RNA-binding protein that plays a role in intracellular RNA transport and in the regulation of translation of target mRNAs. Associated with polysomes. May play a role in the transport of mRNA from the nucleus to the cytoplasm. Binds strongly to poly(G), binds moderately to poly(U) but shows very little binding to poly(A) or poly(C).,miscellaneous:RNA-binding activity is inhibited by RANBP9.,miscellaneous:The mechanism of the severe phenotype in the Asn-304 patient lies in the sequestration of bound mRNAs in nontranslatable mRNP particles. In the absence of FMRP, these same mRNAs may be partially translated via alternate mRNPs, although perhaps abnormally localized or regulated, resulting in typical fragile X syndrome. Asn-304 mutation maps to a position within the second KH domain of FMRP that is critical for stabilizing sequence-specific RNA-protein interactions. Asn-304 mutation abrogates the association of the FMRP KH 2 domain with its target, kissing complex RNA.,PTM:Phosphorylated on several serine residues.,similarity:Belongs to the FMR1 family.,similarity:Contains 1 KH domain.,similarity:Contains 2 KH domains.,subunit:Homooligomer. Found in a RNP granule complex with IGF2BP1. Directly interacts with SMN and TDRD3. Interacts with the SMN core complex that contains SMN1, SIP1/GEMIN2, DDX20/GEMIN3, GEMIN4, GEMIN5, GEMIN6, GEMIN7, GEMIN8 and STRAP/UNRIP. Interacts with FXR1, FXR2, IGF2BP1, NUFIP1, NUFIP2, MCRS1 and RANBP9. Interacts with CYFIP1 and CYFIP2.,tissue specificity:Highest levels found in neurons, brain, testis, placenta and lymphocytes. Also expressed in epithelial tissues and at very low levels in glial cells.,

Tech Support

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Datasheet, COA, SDS, and protocol files can be requested from technical support.

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Storage & Handling

Storage
-15°C to -25°C/1 year(Do not lower than -25°C)
06

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Keywords:Rabbit mAbs