| Clone | RT97 | 
| Concentration | PleaserefertotheCertificateofAnalysisforthelot-specificconcentration. | 
| Host | Mouse | 
| Specificity | ThecloneRT97recognizesthe200kDaneurofilamentpolypeptideinanumberofmammalianandavianspecies.Tumoursspecificallyrecognizedincludephaeochromocytoma,paraganglioma,ganglioneuroblastomaandothertumoursofneuronalorigin. 
 KNOWNSPECIESCROSSREACTIVITY:Recognizesrat,chicken,pigandreptile
 | 
| Isotype | IgG1 | 
| SpeciesReactivity | AvianChickenHumanMammalsPigRatReptile
 | 
| AntibodyType | MonoclonalAntibody | 
| EntrezGeneNumber |  | 
| GeneSymbol |  | 
| UniProtNumber |  | 
| UniProtSummary | FUNCTION:SwissProt:P12036#Neurofilamentsusuallycontainthreeintermediatefilamentproteins:L,M,andHwhichareinvolvedinthemaintenanceofneuronalcaliber.NF-HhasanimportantfunctioninmatureaxonsthatisnotsubservedbythetwosmallerNFproteins. SIZE:1026aminoacids;112480Da
 PTM:ThereareanumberofrepeatsofthetripeptideK-S-P,NFHisphosphorylatedonanumberoftheserinesinthismotif.ItisthoughtthatphosphorylationofNFHresultsintheformationofinterfilamentcrossbridgesthatareimportantinthemaintenanceofaxonalcaliber.&Phosphorylationseemstoplayamajorroleinthefunctioningofthelargerneurofilamentpolypeptides(NF-MandNF-H),thelevelsofphosphorylationbeingaltereddevelopmentallyandcoincidentwithachangeintheneurofilamentfunction.
 DISEASE:SwissProt:P12036#DefectsinNEFHareacauseofsusceptibilitytoamyotrophiclateralsclerosis(ALS)[MIM:105400].ALSisaneurodegenerativedisorderaffectingupperandlowermotorneurons,andresultinginfatalparalysis.Sensoryabnormalitiesareabsent.Deathusuallyoccurswithin2to5years.Theetiologyislikelytobemultifactorial,involvingbothgeneticandenvironmentalfactors.
 SIMILARITY:SwissProt:P12036##Belongstotheintermediatefilamentfamily.
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