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商品描述
Description | |
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CatalogueNumber | AG231 |
BrandFamily | Chemicon® |
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Description | Neurofilament70kDa,porcine |
Overview | Purifiedporcineaxonalneurofilament70(lowmolecularweight,NF-L). |
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Presentation | Liquidin6MUrea,10mMPhosphate,pH7.5. |
StorageandShippingInformation | |
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StorageConditions | Maintainat-20°Cto-70°Cinundilutedaliquotsforupto6monthsafterdateofreceipt.Avoidrepeatedfreeze/thawcycles. |
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ApplicationNotes | Westernblotting Proteinstandard ELISA RADIoimmunoassay Optimalworkingdilutionmustbedeterminedbytheenduser. |
BIOLOGicalInformation | |
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Concentration | 1mg/mL |
Purity | Containsapproximately90%NF-LandasmallamountofNF-M. |
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UniProtSummary | FUNCTION:SwissProt:P07196#Neurofilamentsusuallycontainthreeintermediatefilamentproteins:L,M,andHwhichareinvolvedinthemaintenanceofneuronalcaliber. SIZE:543aminoacids;61517Da DOMAIN:SwissProt:P07196Theextramassandhighchargedensitythatdistinguishtheneurofilamentproteinsfromallotherintermediatefilamentproteinsareduetothetailpieceextensions.Thisregionmayformachargedscaffoldingstructuresuitableforinteractionwithotherneuronalcomponentsorions. PTM:O-glycosylated(Bysimilarity). DISEASE:SwissProt:P07196#DefectsinNEFLarethecauseofCharcot-Marie-Toothdiseasetype1F(CMT1F)[MIM:607734].CMT1FisaformofCharcot-Marie-Toothdisease,themostcommoninheriteddisorderoftheperipheralnervoussystem.Charcot-Marie-Toothdiseaseisclassifiedintwomaingroupsonthebasisofelectrophysiologicpropertiesandhistopathology:primaryperipheraldemyelinatingneuropathyorCMT1,andprimaryperipheralaxonalneuropathyorCMT2.NeuropathiesoftheCMT1grouparecharacterizedbyseverelyreducednerveconductionvelocities(lessthan38m/sec),segmentaldemyelinationandremyelinationwithonionbulbformationsonnervebiopsy,slowlyprogressivedistalmuscleatrophyandweakness,absentdeeptendonreflexes,andhollowfeet.CMT1Fischaracterizedbyonsetininfancyorchildhood(range1to13years).&DefectsinNEFLarethecauseofCharcot-Marie-Toothdiseasetype2E(CMT2E)[MIM:607684].CMT2EisanautosomaldominantformofCharcot-Marie-Toothdiseasetype2.NeuropathiesoftheCMT2grouparecharacterizedbysignsofaxonalregenerationintheabsenceofobviousmyelinalterations,normalorslightlyreducednerveconductionvelocities,andprogressivedistalmuscleweaknessandatrophy. SIMILARITY:Belongstotheintermediatefilamentfamily. MISCELLANEOUS:NF-Listhemostabundantofthethreeneurofilamentproteinsand,astheothernonepithelialintermediatefilamentproteins,itcanformhomopolymeric10-nmfilaments. |
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