产品订购信息：
英文名称  Anti-Prion protein PrP/CD230 

中文名称   朊蛋白CD230抗体说明书 

别    名  AltPrP; ASCR; atal familial insomnia; CD230; CD230 antigen; CJD; Creutzfeld Jakob disease; Gerstmann-Strausler-Scheinker syndrome; GSS; KURU; Major prion protein; PRIO_HUMAN.

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, Horse 

产品类型   一抗  

研究领域    细胞生物 神经生物学 干细胞 细菌及 细胞表面分子

蛋白分子量  predicted molecular weight: 25kDa

性    状   Lyophilized or Liquid

免 疫 原 KLH conjugated synthetic peptide derived from human Prion protein PrP/CD230 

亚    型   IgG

纯化方法   affinity purified by Protein A

储 存 液   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

朊蛋白CD230抗体说明书 产品应用    WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存条件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

产品介绍 The function of PrP is still under debate. May play a role in neuronal development and synaptic plasticity. May be required for neuronal myelin sheath maintenance. May play a role in iron uptake and iron homeostasis (By similarity). Isoform 2 may act as a growth suppressor by arresting the cell cycle at the G0/G1 phase. Soluble oligomers are toxic to cultured neuroblastoma cells and induce apoptosis (in vitro).

Function : The function of PrP is still under debate. May play a role in neuronal development and synaptic plasticity. May be required for neuronal myelin sheath maintenance. May play a role in iron uptake and iron homeostasis (By similarity). Isoform 2 may act as a growth suppressor by arresting the cell cycle at the G0/G1 phase. Soluble oligomers are toxic to cultured neuroblastoma cells and induce apoptosis (in vitro).

Subunit : Monomer and homodimer. Has a tendency to aggregate into amyloid fibrils containing a cross-beta spine, formed by a steric zipper of superposed beta-strands. Soluble oligomers may represent an intermediate stage on the path to fibril formation. Copper binding may promote oligomerization. Interacts with GRB2, APP, ERI3/PRNPIP and SYN1. Mislocalized cytosolically exposed PrP interacts with MGRN1; this interaction alters MGRN1 subcellular location and causes lysosomal enlargement (By similarity). Interacts with KIAA1191.

Subcellular Location : Cell membrane. Golgi apparatus and Cytoplasm. Nucleus. Accumulates outside the secretory route in the cytoplasm, from where it relocates to the nucleus.

Isoform 2 is sumoylated by SUMO1.

DISEASE : Note=PrP is found in high quantity in the brain of humans and animals infected with neurodegenerative diseases known as transmissible spongiform encephalopathies or prion diseases, like: Creutzfeldt-Jakob disease (CJD), fatal familial insomnia (FFI), Gerstmann-Straussler disease (GSD), Huntington disease-like type 1 (HDL1) and kuru in humans; scrapie in sheep and goat; bovine spongiform encephalopathy (BSE) in cattle; transmissible mink encephalopathy (TME); chronic wasting disease (CWD) of mule deer and elk; feline spongiform encephalopathy (FSE) in cats and exotic ungulate encephalopathy (EUE) in nyala and greater kudu. The prion diseases illustrate three manifestations of CNS degeneration: (1) infectious (2) sporadic and (3) dominantly inherited forms. TME, CWD, BSE, FSE, EUE are all thought to occur after consumption of prion-infected foodstuffs.

Defects in PRNP are the cause of Creutzfeldt-Jakob disease (CJD) [MIM:123400]. CJD occurs primarily as a sporadic disorder (1 per million), while 10-15% are familial. Accidental transmission of CJD to humans appears to be iatrogenic (contaminated human growth hormone (HGH), corneal transplantation, electroencephalographic electrode implantation, etc.). Epidemiologic studies have failed to implicate the ingestion of infected annimal meat in the pathogenesis of CJD in human. The triad of microscopic features that characterize the prion diseases consists of (1) spongiform degeneration of neurons, (2) severe astrocytic gliosis that often appears to be out of proportion to the degree of nerve cell loss, and (3) amyloid plaque formation. CJD is characterized by progressive dementia and myoclonic seizures, affecting adults in mid-life. Some patients present sleep disorders, abnormalities of high cortical function, cerebellar and corticospinal disturbances. The disease ends in death after a 3-12 months illness.

Defects in PRNP are the cause of fatal familial insomnia (FFI) [MIM:600072]. FFI is an autosomal dominant disorder and is characterized by neuronal degeneration limited to selected thalamic nuclei and progressive insomnia.

Defects in PRNP are the cause of Gerstmann-Straussler disease (GSD) [MIM:137440]. GSD is a heterogeneous disorder and was defined as a spinocerebellar ataxia with dementia and plaquelike deposits. GSD incidence is less than 2 per 100 million live births. Defects in PRNP are the cause of Huntington disease-like type 1 (HDL1) [MIM:603218]. HDL1 is an autosomal dominant, early onset neurodegenerative disorder with prominent psychiatric features.

Defects in PRNP are the cause of kuru (KURU) [MIM:245300]. Kuru is transmitted during ritualistic cannibalism, among natives of the New Guinea highlands. Patients exhibit various movement disorders like cerebellar abnormalities, rigidity of the limbs, and clonus. Emotional lability is present, and dementia is conspicuously absent. Death usually occurs from 3 to 12 month after onset.

Defects in PRNP are the cause of spongiform encephalopathy with neuropsychiatric features (SENF) [MIM:606688]; an autosomal dominant presenile dementia with a rapidly progressive and protracted clinical course. The dementia was characterized clinically by frontotemporal features, including early personality changes. Some patients had memory loss, several showed aggressiveness, hyperorality and verbal stereotypy, others had parkinsonian symptoms.

Prion diseases, or transmissible spongiform encephalopathies (TSEs), are manifested as genetic, infectious or sporadic, lethal neurodegenerative disorders involving alterations of the prion protein (PrP). Characteristic of prion diseases, cellular PrP (PrPc) is converted to the disease form, PrPSc, through alterations in the protein folding conformations. PrPc is constitutively expressed in normal adult brain and is sensitive to proteinase K digestion, while the altered PrPSc conformation is resistant to proteases, resulting in a distinct molecular mass after PK treatment. Consistent with the transient infection process of prion diseases, incubation of PrPc with PrPSc both in vitro and in vivo produces PrPc that is resistant to protease degradation. Infectious PrPSc is found at high levels in the brains of animals affected by TSEs, including scrapie in sheep, BSE in cattle and Cruetzfeldt-Jakob disease in humans.

Similarity : Belongs to the prion family.

Database links : UniProtKB/Swiss-Prot: P04156.1

25(OH)D3/25 HVD3(Human 25-Dihydroxy vitamin D3)ELISA Kit 人25羟基维生素D3Multi-class antibodies规格： 48T

Anti-MMP-16 基质金属蛋白酶-16抗体Multi-class antibodies规格： 0.1ml

Rhesus antibody Rh Interferon alpha 6 干扰素α6抗体 规格 0.2ml

anti-HAV(Human anti-hepatitis A virus IgG antibody) ELISA Kit 人IgG抗体 96T

RNA polymerase II 英文名称： RNA聚合酶II抗体 0.2ml

Alx1 英文名称： 软骨蛋白1抗体 0.1ml

Anti-MMP-16 基质金属蛋白酶-16抗体Multi-class antibodies规格： 0.1ml

NO(Mouse nitric oxide) ELISA Kit 小鼠Multi-class antibodies规格： 48T

Anti-Chloramphenicol/FITC 荧光素标记抗氯霉素抗体IgGMulti-class antibodies规格： 0.2ml

Rhesus antibody Rh Dog IgM/Cy7 Cy7标记的兔抗犬IgM抗体 规格 0.1ml

Rat IgM/RBITC 罗丹明标记大鼠IgM 0.3ml

Lamin B 英文名称： 核纤层蛋白B抗体(细胞核膜标志物) 0.1ml

磺胺类多残留快速检测卡 50T/盒 蜂蜜、奶、奶粉

Anti-Chloramphenicol/FITC 荧光素标记抗氯霉素抗体IgGMulti-class antibodies规格： 0.2ml

Anti-IL-17D/IL-27/FITC 荧光素标记白介素-17D抗体IgGMulti-class antibodies规格： 0.2ml

Anti-RAMP-1/FITC 荧光素标记受体活性调制蛋白1抗体IgGMulti-class antibodies规格： 0.2ml

Rhesus antibody Rh APBB1/Fe65 protein 铁蛋白Fe65抗体 规格 0.1ml

Rabbit Anti-rat IgG/Cy7 Cy7标记的兔抗大鼠IgG 0.1ml

GIMAP2 英文名称： GTP酶IMAP家族成员2抗体 0.2ml

Rhesus antibody Rh Rabbit Anti-human IgM/Cy7 Cy7标记的兔抗人IgM 规格 0.1ml

Anti-RAMP-1/FITC 荧光素标记受体活性调制蛋白1抗体IgGMulti-class antibodies规格： 0.2ml

大鼠抗激素/加压素/精酸加压素(ADH/VP/AVP)ELISA试剂盒 ，英文名： ADH/VP/AVP ELISA Kit

人样生长因子结合蛋白3(IGFBP-3)ELISA检测试剂盒Humaninsulin-likegrowthfactorsbindingprotein3,IGFBP-3ELISAKit 96T/48T

新城疫病毒(NDV)核酸检测试剂盒(PCR-荧光探针法) 48T

CLIAKitforNP-Y(MouseneuropeptideY)ELISAKit小鼠神经肽Y规格：48T/96T

体外非细胞系统细胞色素P450亚酶CYP2B(EFC)活性荧光定量检测试剂盒20次

ELISAKitα-SCA人α横纹肌肌动蛋白规格：48T/96T

大鼠白介素22受体α2(IL2Rα2)ELISA试剂盒 ，英文名： IL2Rα2 ELISA Kit

Human beta lactose protein aibody IgGELISA Kit 人β乳糖蛋白抗体IgGELISA试剂盒

Sophorajaponicaagglinin,SJAELISAKit 槐凝集素(SJA)ELISA试剂盒 96T/48T 进口分装

CLIAKitforCLA(HumanCollagenaoaibody)ELISAKit人抗胶原蛋白抗体规格：48T/96T

细胞脂酰辅酶A合成酶(ACYLCOASYHETASE)总活性酶连续循环比色法定量检测试剂盒20次

RatCalcitonin,CTELISAKit大鼠降钙素(CT)ELISA试剂盒规格：96T/48T

朊蛋白CD230抗体说明书 大鼠成纤维细胞生长因子11(FGF11)ELISA试剂盒 ，英文名： FGF11 ELISA Kit

Human XC chemokine receptor 1 (XCR1) ELISA Kit 人XC趋化因子受体1(XCR1)ELISA试剂盒

RatCarboxyterminalpropeptideoftypeⅠprocollagen,PⅠCPELISAKit 大鼠Ⅰ型前胶原羧基端肽(PⅠCP)ELISA试剂盒 96T/48T 进口分装

CLIAKitforCr(MouseCrosslaps)ELISAKit小鼠骨胶原交联规格：48T/96T

细胞紧张素Ⅰ转化酶2(ACE2)活性荧光共振能量转移法(FRET)定量检测试剂盒20次

RatCystatinC,Cys-CELISAKit大鼠半胱氨酸蛋白酶抑制剂/胱抑素C(Cys-C)ELISA试剂盒规格：96T/48T

抗体的生物素化标记实验要点：

1. 朊蛋白CD230抗体说明书 如在反应混合液中有叠氮钠或游离氨基存在,会抑制标记反应。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硼酸缓冲液充分透析；

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最适条件；

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。

抗体的鉴定：

1）朊蛋白CD230抗体说明书 抗体的效价鉴定：不管是用于诊断还是用于,制备抗体的目的都是要求较高效价。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抑制试验测定。以不同浓度抗原和近似抗原分别做竞争抑制曲线,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。