Clone | RT97 |
Concentration | PleaserefertotheCertificateofAnalysisforthelot-specificconcentration. |
Host | Mouse |
Specificity | ThecloneRT97recognizesthe200kDaneurofilamentpolypeptideinanumberofmammalianandavianspecies.Tumoursspecificallyrecognizedincludephaeochromocytoma,paraganglioma,ganglioneuroblastomaandothertumoursofneuronalorigin.
KNOWNSPECIESCROSSREACTIVITY:Recognizesrat,chicken,pigandreptile |
Isotype | IgG1 |
SpeciesReactivity | - Avian
- Chicken
- Human
- Mammals
- Pig
- Rat
- Reptile
|
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. |