4000-520-616
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主营:主营:血管内皮生长因子-C, Spp1, IRF-3, DPYSL2, GLUT5
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当前位置: 首页 > 产品中心 > Other_kits > IBL/苯丙氨酸(PKU)新生儿筛查试验(480检测)/RE80015/
商品详细IBL/苯丙氨酸(PKU)新生儿筛查试验(480检测)/RE80015/
IBL/苯丙氨酸(PKU)新生儿筛查试验(480检测)/RE80015/
IBL/苯丙氨酸(PKU)新生儿筛查试验(480检测)/RE80015/
商品编号: RE80015
品牌: TECAN
市场价: ¥0.00
美元价: 0.00
产地: 美国(厂家直采)
公司:
产品分类: 其它检测试剂盒
公司分类: Other_kits
联系Q Q: 3392242852
电话号码: 4000-520-616
电子邮箱: info@ebiomall.com
商品介绍
Kitsize480determinations
MethodEnzymaticassay
Incubationtime2x30min,1x3min
Standardrange1-18mg/dL
Specimen/Volumesbloodspot
Substrate/isotopeINT570nm
RegulatoryStatus:EU:CE
Detailsfor: Phenylalanin(PKU)NeonatalScreeningAssay(480det.)
EnzymaticAssayforthein-vitro-diagnosticquantitativedeterminationofL-Phenylalanineinhumannewbornbloodspots.ForneonatalscreeningonPhenylketonuria.

Phenylketonuria(PKU)isoneofthemostoftenheriditarydiseasesofthemetabolismoftheaminoacids.It
istransmittedautosomalrecessivetothedescendantwithanincidenceofcirca1:2600to1:25000
dependentontheobservedpopulationgroup.Themaincauseofthedisease–90to99%ofallcases–isadecreaseortheabsenceoftheactivityoftheenzymecomplexPhenylalaninehydroxylasewhichisresponsIBLeforthetransformationoftheessentialaminoacidPhenylalanineintoTyrosine.ThelatterisaprecursoroftheCatecholamines,Melanineandthethyroidhormones.BecauseoftheblockingofthismetabolismPhenylalanineistransformedbyanalternativepathwaytoPhenylpyruvateand–acetatewhichareexcretedbythekidneys.Thediseasebecomesapparentbyamentalretardationofthepatientsbeginninginthefirstweeksoftheirlife.Responsibleforthisdevelopmentistheimpropermyelinizationoftheneuronsinthebrainbecauseofthechangeintheproteinmetabolism.OthersignsofthePhenylketonuriaarethelackingofpigmentationofthedermisanditsadnexisbecauseofthedisturbanceofthesynthesisofMelanineandthereforeitspredispositionofskindiseases.ItisimportanttomakethediagnosisofPhenylketonuriainthenewbornsbecausethecerebraldamagecanbepreventedbyalowPhenylalaninediet.ThereforeascreeningtestforthedetectionofelevatedPhenylalanineconcentrationsinthebloodhastobemadebetweenthe2rdandthe5thdayoftheinfantslife.Ifthisoneispositiveitisfollowedbyaconfirmatoryassaytodetectthespecialmutationsonthechromosome12.ManyscreeningtestsweredevelopedformonitoringpatientsforPhenylketonuria.ThefirstonecalledtoitsinventorGUTHRIEisbasedontheneutralizationofangrowthinhibitingfactorofBacillussubtilisbyhighPhenylalaninelevels.BecauseofsomedisadvantagesofthismethoditisreplacedbyassaysinwhichthePhenylalanineinthebloodofthepatientstakespartinachemicalreactiondevelopingafluorescinatingoracolouredsubstancewhichcanbemeasuredquantitavely.TheprocedurescanbeusedmoreconvenientlyincomparisontothemoresophisticatedandmoreexpensivemethodoftheHPLC.
ForconcretedatapleaseconsulttheInstructionforUseinthedownloadboxontherightside.
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