心肌兰尼碱受体抗体（脑肌兰尼碱受体）说明书

货号： EY-2150K 保存条件：  Store at -20 °C 浓度： 1mg/1ml 应用范围： 产品应用ELISA=1:500-1000IHC-P=1:100-500IHC-F=1:100-500IF=1:100-500 形态： 详见说明书 亚型：  IgG   免疫原： KLHconjugatedsyntheticpeptidederivedfromhumanRyanodineReceptor 适应物种： 详见说明书 宿主： 详见说明书 标记物： 详见说明书 抗体名： 心肌兰尼碱受体抗体（脑肌兰尼碱受体）说明书 抗体英文名： Anti-RyanodineReceptor 保质期： 详见说明书 抗原来源： Rabbit 克隆性： 多克隆 目录编号： 详见说明书 级别： 详见说明书 靶点： 详见说明书 数量： 60 供应商： 上海一研 规格： 0.2ml/200μg

心肌兰尼碱受体抗体（脑肌兰尼碱受体）说明书英文名称  Anti-RyanodineReceptor
中文名称 心肌兰尼碱受体抗体（脑肌兰尼碱受体）说明书
别    名 Arrhythmogenicrightventriculardysplasia2;ARVC2;ARVC2;ARVD2;ARVD2;Brainryanodinereceptorcalciumreleasechannel;Braintyperyanodinereceptor;Cardiacmuscleryanodinereceptor;Cardiacmuscleryanodinereceptorcalciumreleasechannel;Cardiacmuscletyperyanodinereceptor;CCO;Centralcorediseaseofmuscle;HBRR;hRYR2;hRYR2;MHS;MHS1;Ryanodinereceptor1(skeletal);Ryanodinereceptor1;Ryanodinereceptor2(cardiac);Ryanodinereceptor2;Ryanodinereceptor3;Ryanodinereceptortype1;RYDR;RYR1;RYR2;RYR3;RYR;RYR1;RYR2;RYR3;Sarcoplasmicreticulumcalciumreleasechannel;Skeletalmusclecalciumreleasechannel;Skeletalmuscleryanodinereceptor;Skeletalmuscletyperyanodinereceptor;SKRR;Type1likeryanodinereceptor;VTSIP.

浓度1mg/1ml
规格0.2ml/200μg
抗体来源Rabbit
克隆类型polyclonal
交叉反应Human,Mouse,Rat,Dog,Pig,Cow,Rabbit
产品类型一抗
研究领域肿瘤心血管通道蛋白细胞膜受体
蛋白分子量predictedmolecularweight：
566kDa 
免疫原KLHconjugatedsyntheticpeptidederivedfromhumanRyanodineReceptor
亚型IgG
纯化方法affinitypurifiedbyProteinA
储存液Preservative:15mMSodiumAzide,Constituents:1%BSA,0.01MPBS,pH7.4
产品应用ELISA=1:500-1000IHC-P=1:100-500IHC-F=1:100-500IF=1:100-500
（石蜡切片需做抗原修复）
notyettestedinotherapplications.
optimaldilutions/concentrationsshouldbedeterminedbytheenduser.
保存条件Storeat-20°Cforoneyear.Avoidrepeatedfreeze/thawcycles.Thelyophilizedantibodyisstableatroomtemperatureforatleastonemonthandforgreaterthanayearwhenkeptat-20°C.WhenreconstitutedinsterilepH7.40.01MPBSordiluentofantibodytheantibodyisstableforatleasttwoweeksat2-4°C.
ImportantNoteThisproductassuppliedisintendedforresearchuseonly,notforuseinhuman,therapeuticordiagnosticapplications.
产品介绍TheRyanodineReceptor(RyR)isthechannelresponsibleforcalciumreleasefrommusclecellSarcoplasmicReticulum(SR)andalsoplaysaroleincalciumregulationinnon-musclecells.TheRyRexistsasahomotetramerandispredictedtohaveashortcytoplasmicC-terminusand4-10transmembranedomains.Theremainderoftheprotein,termedthe"foot"region,islocatedinthecytoplasmbetweenthetransversetubuleandtheSR.MammalianRyRisoformsaretheproductofthreedifferentgenes：
RyR-1isexpressedpredominantlyinskeletalmuscleandareasofthebrain;RyR-2isexpressedpredominantlyinheartmusclebutalsofoundinthestomach,endothelialcellsanddiffuseareasofthebrain;andRyR-3isfoundinsmoothmuscleandthebrain(striatum,thalamusandhippocampus).Innon-mammalianvertebrates,theRyRisoformsaretermedalpha,betaandcardiacwhichcorrelatelooselytothemammalianRyR-1,RyR-3andRyR-2isoformsrespectively. 
Function：
CalciumchannelthatmediatesthereleaseofCa(2+)fromthesarcoplasmicreticulumintothecytoplasmandtherebyplaysakeyroleintriggeringmusclecontractionfollowingdepolarizationofT-tubules.Repeatedveryhigh-levelexerciseincreasestheopenprobabilityofthechannelandleadstoCa(2+)leakingintothecytoplasm.CanalsomediatethereleaseofCa(2+)fromintracellularstoresinneurons,andmaytherebypromoteprolongedCa(2+)signalinginthebrain.Requiredfornormalembryonicdevelopmentofmusclefibersandskeletalmuscle.Requiredfornormalheartmorphogenesis,skindevelopmentandossificationduringembryogenesis(Bysimilarity). 
Subunit：
Homotetramer.CanalsoformheterotetramerswithRYR2.InteractswithCALM;CALMwithboundcalciuminhibitstheRYR1channelactivity.InteractswithS100A1.InteractswithFKBP1A;thisstabilizestheclosedconformationofthechannel.InteractswithCACNA1S;interactionwithCACNA1SisimportantforactivationoftheRYR1channel.InteractswithCACNB1.InteractswithTRDNandASPH;theseinteractionsstimulateRYR1channelactivity(Bysimilarity).IdentifiedinacomplexcomposedofRYR1,PDE4D,PKA,FKBP1Aandproteinphosphatase1(PP1).Repeatedveryhigh-levelexercisedecreasesinteractionwithPDE4Dandproteinphosphatase1(PP1). 
SubcellularLocation：
Sarcoplasmicreticulummembrane;Multi-passmembraneprotein(Probable).Membrane;Multi-passmembraneprotein.Microsomemembrane;Multi-passmembraneprotein. 
TissueSpecificity：
Brain,skeletalmuscle,placentaandpossiblyliverandkidney.Inbrain,highestlevelsarefoundinthecerebellum,hippocampus,caudatenucleusandamygdala,withlowerlevelsinthecorpuscallosum,substantianigraandthalamus. 
Post-translationalmodifications：
Channelactivityismodulatedbyphosphorylation.PhosphorylationatSer-2843mayincreasechannelactivity.Repeatedveryhigh-levelexerciseincreasesphosphorylationatSer-2843.[PTM]ActivatedbyreversibleS-nitrosylation.Repeatedveryhigh-levelexerciseincreasesS-nitrosylation. 
DISEASE:Malignanthyperthermia1(MHS1)[MIM:145600]:Autosomaldominantpharmacogeneticdisorderofskeletalmuscleandisoneofthemaincausesofdeathduetoanesthesia.Insusceptiblepeople,anMHepisodecanbetriggeredbyallcommonlyusedinhalationalanestheticssuchashalothaneandbydepolarizingmusclerelaxantssuchassuccinylcholine.Theclinicalfeaturesofthemyopathyarehyperthermia,acceleratedmusclemetabolism,contractures,metabolicacidosis,tachycardiaanddeath,ifnottreatedwiththepostsynapticmusclerelaxant,dantrolene.SusceptibilitytoMHcanbedeterminedwiththe'invitro'contracturetest(IVCT):observingthemagnitudeofcontracturesinducedinstripsofmuscletissuebycaffeinealoneandhalothanealone.PatientswithnormalresponseareMHnormal(MHN),thosewithabnormalresponsetocaffeinealoneorhalothanealoneareMHequivocal(MHE(C)andMHE(H)respectively).Note=Thediseaseiscausedbymutationsaffectingthegenerepresentedinthisentry.
Centralcorediseaseofmuscle(CCD)[MIM:117000]:Autosomaldominantcongenitalmyopathy,butasevereautosomalrecessiveformalsoexists.Bothclinicalandhistologicalvariabilityisobserved.Affectedindividualstypicallydisplayhypotoniaandproximalmuscleweaknessininfancy,leadingtothedelayofmotormilestones.Theclinicalcourseofthedisorderisusuallyslowornonprogressiveinadulthood,andtheseverityofthesymptomsmayvaryfromnormaltosignificantmuscleweakness.MicroscopicexaminationofCCD-affectedskeletalmusclerevealsapredominanceoftypeIfiberscontaininrphous-lookingareas(cores)thatdonotstainwithoxidativeandphosphorylasehistochemicaltechniques.Note=Thediseaseiscausedbymutationsaffectingthegenerepresentedinthisentry.
Multiminicorediseasewithexternalophthalmoplegia(MMDO)[MIM:255320]:Clinicallyheterogeneousneuromusculardisorder.Generalfeaturesincludeneonatalhypotonia,delayedmotordevelopment,andgeneralizedmuscleweaknessandamyotrophy,whichmayprogressslowlyorremainstable.Musclebiopsyshowsmultiple,poorlycircumscribed,shortareasofsarcomeredisorganizationandmitochondriadepletion(areastermedminicores)inmostmusclefibers.Typically,nodystrophicsigns,suchasmusclefibernecrosisorregenerationorsignificantendomysialfibrosis,arepresentinmultiminicoredisease.Note=Thediseaseiscausedbymutationsaffectingthegenerepresentedinthisentry.
Congenitalmyopathywithfiber-typedisproportion(CFTD)[MIM:255310]:Geneticallyheterogeneousdisorderinwhichthereisrelativehypotrophyoftype1musclefiberscomparedtotype2fibersonskeletalmusclebiopsy.However,thesefindingsarenotspecificandcanbefoundinmanydifferentmyopathicandneuropathicconditions.Note=Thediseaseiscausedbymutationsaffectingthegenerepresentedinthisentry.
Note=DefectsinRYR1maybeacauseofSamaritanmyopathy,acongenitalmyopathywithbenigncourse.Patientsdisplayseverehypotoniaandrespiratorydistressatbirth.Unlikeothercongenitalmyopathies,thehealthstatusconstantlyimprovesandpatientsareminimallyaffectedatadulthood.
Similarity：
Belongstotheryanodinereceptor(TC1.A.3.1)family.RYR3subfamily. 
Contains3B30.2/SPRYdomains.
Contains5MIRdomains.
Databaselinks:UniProtKB/Swiss-Prot:P21817.3
DefectsintheRYR2genearethecauseofseveralheartdiseases：
1)familialarrhythmogenicrightventriculardysplasia2(ARVD2),alsoknownasarrhythmogenicrightventricularcardiomyopathy2(ARVC2),2)anautosomaldominantformofstress-inducedpolymorphicventriculartachycardia(VTSIP),alsoknownascatecholaminergicpolymorphicventriculartachycardia(CPVT)and3)familialpolymorphicventriculartachycardia(FPVT).RyanodineReceptor2containsseveralphosphorylatablesites.Specifically,Ser-2030andSer-2809(oratSer-2808dependingonthespecies)canbephosphoryatedbyproteinkinaseA(PKA)andSer-2815(oratSer-2814dependingonthespecies)canbephosphorylatedbyCaMKII(Ca2+/calmodulin-dependentproteinkinaseII). 

心肌兰尼碱受体抗体（脑肌兰尼碱受体）说明书技术外包服务:
★分子生物学：质粒抽提、PCR、Q-PCR、RT-PCR、分子生物学：基因合成、引物合成、基因测序、载体构建等
★蛋白工程：原核、哺乳动物蛋白表达系统等
★病毒包装：腺病毒、慢病毒等
★抗体工程：磁珠分选、病理染色、WB、ELISA、IP、IF、IHC、FACS、Confocal等等
★细胞工程：细胞表型分析(凋亡、增殖、周期、迁移、侵袭、修复、克隆形成)、细胞培养、细胞膜制备、稳定细胞株构建、细胞RNAi技术等等。
心肌兰尼碱受体抗体（脑肌兰尼碱受体）说明书抗体的生物素化标记实验要点：
1.如在反应混合液中有叠氮钠或游离氨基存在,会YZ标记反应。因此,蛋白质在反应前要对 0.1mol/L碳酸氢钠缓冲液或0.5mol/L缓冲液充分透析；
2.所用的NHSB及待生物素化蛋白质之间的分子比按蛋白质表面的ε-氨基的密度会有所不同,选择不当则影响标记的效率,应先用几个不同的分子比来筛选Z适条件；
3.用NHSB量过量也是不利的,抗原的结合位点可能因此被封闭,导致抗体失活；
4.由于抗体的氨基不易接近可能造成生物素化不足,此时可加入去污剂如 Triton x-100, Tween20等；
5.当游离ε-氨基(赖氨酸残基的氨基)存在于抗体的抗原结合位点时,或位于酶的催化位点时,生物素化会降低或损伤抗体蛋白的结合力或活性；
6.生物素还可能与不同的功能基团,如羰基、氨基、巯基、异咪唑基及基,也可与糖基共价结合；
7.交联反应后,应充分透析,否则,残余的生物素会对生物素化抗体与亲和素的结合产生竞争作用；
8.在细胞的荧光标记实验中,中和亲和素的本底低,但由于链霉亲和素含有少量正电荷,故对某些细胞可导致高本底。
ELISA Kit for Human Serpin B40.156-10 ng/mL
人α2-纤溶酶YZ因子ELISA试剂盒62.5-4000 pg/mL
ELISA Kit for Human Alpha-2-antiplasmin62.5-4000 pg/mL
人S100钙结合蛋白A16ELISA试剂盒0.312-20 ng/mL
ELISA Kit for Human Protein S100-A160.312-20 ng/mL
人P选择素(P-Selectin/CD62P/GMP140)ELISA试剂盒62.5-4000 pg/mL
ELISA Kit for Human P-selectin62.5-4000 pg/mL
人L选择素(L-Selectin/CD62L)ELISA试剂盒0.78-50 ng/ml
ELISA Kit for Human L-selectin0.78-50 ng/ml
人E选择素(E-Selectin/CD62E)ELISA试剂盒0.156-10 ng/mL
ELISA Kit for Human E-selectin0.156-10 ng/mL
四氢生物蝶呤(Tetrahydrobiopterin)ELISA试剂盒0.78-50 uIU/mL
ELISA Kit for Tetrahydrobiopterin0.78-50 uIU/mL
甘油三脂(Triglyceride)ELISA试剂盒0.312-20 nmol/mL
ELISA Kit for Triglyceride0.312-20 nmol/mL
血栓素A2(Thromboxane A2)ELISA试剂盒3.12-200 pg/mL
ELISA Kit for Thromboxane A23.12-200 pg/mL
血栓素B2(Thromboxane B2)ELISA试剂盒15.6-1000 pg/mL
ELISA Kit for Thromboxane B215.6-1000 pg/mL
睾酮(T)ELISA试剂盒0.156-10 ng/mL
心肌兰尼碱受体抗体（脑肌兰尼碱受体）说明书人 VEGFA 转录变体4 基因全长ORF克隆
人 NMNAT2 基因全长ORF克隆
人 MITF 基因全长ORF克隆
人 FAP 基因全长ORF克隆
人 CHST11 基因全长ORF克隆
人 SGPL1 基因全长ORF克隆
人 BMX 转录变体2 基因全长ORF克隆
人 AHSA1 基因全长ORF克隆
人 GAL 基因全长ORF克隆
人 BRIX1 基因全长ORF克隆
人 CAPN5 基因全长ORF克隆
人 FLRT1 基因全长ORF克隆
人 ALDH1A1 基因全长ORF克隆
人 LRRTM4 基因全ᰀ
心肌兰尼碱受体抗体（脑肌兰尼碱受体）说明书抗体的鉴定：
1）抗体的效价鉴定：不管是用于诊断还是用于ZL,制备抗体的目的都是要求较GX价。不同的抗原制备的抗体,要求的效价不一。鉴定效价的方法很多,包括有试管凝集反应,琼脂扩散试验,酶联免疫吸附试验等。常用的抗原所制备的抗体一般都有约成的鉴定效价的方法,以资比较。如制备抗抗体的效价,一般就采用琼脂扩散试验来鉴定。
2）抗体的特异性鉴定：抗体的特异性是指与相应抗原或近似抗原物质的识别能力。抗体的特异性高,它的识别能力就强。衡量特异性通常以交叉反应率来表示。交叉反应率可用竞争YZ试验测定。以不同浓度抗原和近似抗原分别做竞争YZ曲线,计算各自的结合率,求出各自在IC50时的浓度,并按公式计算交叉反应率。 
如果所用抗原浓度IC50浓度为pg/管,而一些近似抗原物质的IC50浓度几乎是无穷大时,表示这一抗血清与其他抗原物质的交叉反应率近似为0,即该血清的特异性较好。
3）抗体亲和力：是指抗体和抗原结合的牢固程度。亲和力的高低是由抗原分子的大小,抗体分子的结合位点与抗原决定簇之间立体构型的合适度决定的。有助于维持抗原抗体复合物稳定的分子间力有氢键,疏水键,侧链相反电荷基因的库仑力,范德华力和空间斥力。亲和力常以亲和常数K表示,K的单位是L/mol。抗体亲和力的测定对抗体的筛选,确定抗体的用途,验证抗体的均一性等均有重要意义。