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| Species | Human |
|---|---|
| SampleType | Plasma.ForthequantitativedeterminationofhumanFibrinogenlevelsinplasma.Blood,VascularDiseases,PathologicProcesses,BloodCells,HematologicDiseases. |
| ProductName | HumanFibrinogen. |
| SampleType | Plasma |
| #ofSamples | 10X96wells |
| StorageandStability | -20°C |
| RangeofDetection | 6.25-400ng/mL |
| Shelflife | Oneyearfromdateofreceipt. |
| AssayCondition | ThekitperformancehasbeenoptimizedfortheprotocolandmaterialslistedbelowusingstandarddilutionsofhumanFibrinogeninthe6.25-400ng/mLrange.Theoperatormustdetermineappropriatedilutionsofreagentsforalternativeassayconditions.ELISAassayreactivityissensitivetovariationsinoperator,pipettingandwashingtechniques,incubationtime,temperature,compositionofreagents,andotherexperimentalvariables.Assayoptimizationmayberequiredtogeneratethestandardcurveandfitthesamplesinthespecifieddetectionrange. |
| CountryofOrigin | UnitedStatesofAmerica. |
| AssayUse | ForResearchUseOnly(RUO).Notfordiagnosticortherapeuticuseinhumansoranimals.Notforanimalorhumanconsumption. |
| Function | Fibrinogenhasadoublefunction:yieldingmonomersthatpolymerizeintofibrinandactingasacofactorinplateletaggregation. |
| Subunit | Heterohexamer;disulfidelinked.Contains2setsof3non-identicalchains(alpha,betaandgamma).The2heterotrimersareinheadtoheadconformationwiththeN-terminiinasmallcentraldomain. |
| SubcellularLocation | Secreted. |
| Domain | Alongcoiledcoilstructureformedby3polypeptidechainsconnectsthecentralnoduletotheC-terminaldomains(distalnodules).ThelongC-terminalendsofthealphachainsfoldback,contributingafourthstrandtothecoiledcoilstructure. |
| Ptm | Thealphachainisnotglycosylated. FormsF13A-mediatedcross-linksbetweenaglutamineandtheepsilon-aminogroupofalysineresidue,formingfibronectin-fibrinogenheteropolymers. Aboutone-thirdofthealphachainsinthemoleculesinbloodwerefoundtobephosphorylated. Conversionoffibrinogentofibrinistriggeredbythrombin,whichcleavesfibrinopeptidesAandBfromalphaandbetachains,andthusexposestheN-terminalpolymerizationsitesresponsiblefortheformationofthesoftclot.ThesoftclotisconvertedintothehardclotbyfactorXIIIAwhichcatalyzestheepsilon-(gamma-glutamyl)lysinecross-linkingbetweengammachains(stronger)andbetweenalphachains(weaker)ofdifferentmonomers. |
| Disease | DefectsinFGAareacauseofcongenitalafibrinogenemia[MIM:202400].Thisisarareautosomalrecessivedisordercharacterizedbybleedingthatvariesfrommildtosevereandbycompleteabsenceorextremelylowlevelsofplasmaandplateletfibrinogen.Themajorityofcasesofafibrinogenemiaareduetotruncatingmutations. VariationsinpositionArg-35(thesiteofcleavageoffibrinopeptideabythrombin)leadstoalpha-dysfibrinogenemias. DefectsinFGAareacauseofamyloidoistype8(AMYL8)[MIM:105200];alsoknownassystemicnon-neuropathicamyloidosisorOstertag-typeamyloidosis.AMYL8isahereditarygeneralizedamyloidosisduetodepositionofapolipoproteinA1,fibrinogenandlysozymeamyloids.Visceraareparticularlyaffected.Thereisnoinvolvementofthenervoussystem.Clinicalfeaturesincluderenalamyloidosisresultinginnephroticsyndrome,arterialhypertension,hepatosplenomegaly,cholestasis,petechialskinrash. |
| Similarity | Contains1fibrinogenC-terminaldomain. |
| Name | HumanFibrinogenELISAQuantitationKit |
|---|---|
| RelatedProductNames | HumanFibrinogenELISAKit;N/AHumanFibrinogenFGA |
| NCBIAccNumber | NP_000499.1 |
| MolecularWeight | 94973 |
| SwissProtNumber | P02671 |
| IntendedUse | ResearchUseOnly |