抗体的生物素化标记实验要点：

1. 肌痉挛相关EPM2蛋白抗体费用 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。

产品订购信息：
英文名称  Anti-NHLRC1/EPM2A 

中文名称   肌痉挛相关EPM2蛋白抗体费用 

别    名  E3 ubiquitin-protein ligase NHLRC1; EPM2A; EPM2B; Malin; MGC119262; MGC119264; NHL repeat containing 1; NHL repeat containing protein 1; NHL repeat-containing protein 1; NHLC1_HUMAN; NHLRC 1; Nhlrc1.

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, Rabbit 

产品类型   一抗  

研究领域    心 细胞生物 免疫学 神经生物学

蛋白分子量  predicted molecular weight: 42kDa

性    状   Lyophilized or Liquid

免 疫 原 KLH conjugated synthetic peptide derived from human NHLRC1 

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

肌痉挛相关EPM2蛋白抗体费用 产品应用    WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-F=1:100-500 IF=1:50-200

（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 Progressive myoclonic epilepsy type 2 (EPM2), also called Lafora disease, is an autosomal recessive disease characterized by grand mal seizures and/or myoclonus at about 15 years of age. Rapid and severe mental deterioration follows, often with psychotic features. Survival is less than 10 years after onset. Starch-like, endoplasmic reticulum-associated polyglucosans, called Lafora bodies, can be observed in brain, muscle, liver and heart. One cause of Lafora disease is due to mutations in NHLRC1, the gene encoding Malin. Forty-nine different mutations in NHLRC1 have been shown to cause EPM2. Malin, also called NHL repeat-containing protein 1, is a single subunit E3 ubiquitin ligase, containing 6 NHL repeats and 1 RING-type zinc finger. Malin’s RING domain is responsible for its ability to mediate ubiquitination. Malin interacts with and polyubiquitinates Laforin, a protein also implicated in EPM2. Malin localizes to the endoplasmic reticulum and, to a lesser extent, in the nucleus. Malin is expressed in brain, cerebellum, spinal cord, medulla, heart, liver, skeletal muscle and pancreas.

Function : E3 ubiquitin-protein ligase which in complex with EPM2A/laforin and HSP70 suppresses the cellular toxicity of misfolded proteins by promoting their degradation through the ubiquitin-proteasome system (UPS). Ubiquitinates PPP1R3C/PTG in a laforin-dependent manner, and targets it for proteasome-dependent degradation and this degradation decreases glycogen accumulation. Polyubiquitinates EPM2A/laforin and ubiquitinates AGL and targets them for proteasome-dependent degradation.Subunit : Interacts with AGL. Interacts (via the NHL repeats) with EPM2A/laforin. Forms a complex with EPM2A/laforin and HSP70.Subcellular Location : Endoplasmic reticulum. Nucleus. Localizes at the endoplasmic reticulum and, to a lesser extent, in the nucleus.Tissue Specificity : Expressed in brain, cerebellum, spinal cord, medulla, heart, liver, skeletal muscle and pancreas.DISEASE : Defects in NHLRC1 are a cause of progressive myoclonic epilepsy type 2 (EPM2) [MIM:254780]; also known as Lafora disease. EPM2 is an autosomal recessive and severe form of adolescent-onset progressive epilepsy. Typically, as seizures increase in frequency, cognitive function declines towards dementia, and affected individuals die usually within 10 years after onset. EPM2 occurs worldwide, but it is particularly common in the mediterranean countries of southern Europe and northern Africa, in southern India and in the Middle East. At the cellular level, it is characterized by accumulation of starch-like polyglucosans called Lafora bodies (LBs) that are most abundant in organs with the highest glucose metabolism: brain, heart, liver and skeletal muscle. Among other conditions involving polyglucosans, EPM2 is unique in that the inclusions are in neuronal dendrites but not axons and the forming polyglucosan fibrils are associated with the endoplasmic reticulum.Similarity : Contains 6 NHL repeats.Contains 1 RING-type zinc finger.Database links : UniProtKB/Swiss-Prot: Q6VVB1.2

抗体的鉴定：

1）肌痉挛相关EPM2蛋白抗体费用 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。

KAF/AR ELISA Kit 大鼠角化细胞因子/双调蛋白Multi-class antibodies规格： 48T

Anti-AR Alpha1/FITC 荧光素标记α1能受体抗体IgGMulti-class antibodies规格： 0.2ml

Rhesus antibody Rh FAM155A FAM155A蛋白抗体 规格 0.2ml

IL-27(human Interleukin 27) ELISA Kit 人白介素27 96T

MARCO 英文名称： 巨噬细胞清道夫受体抗体 0.2ml

Annexin A13 英文名称： 膜粘连蛋白13抗体 0.2ml

Anti-AR Alpha1/FITC 荧光素标记α1能受体抗体IgGMulti-class antibodies规格： 0.2ml

sIgA(Mouse secretory immunoglobulin A) ELISA Kit 小鼠分泌型免疫球蛋白AMulti-class antibodies规格： 48T

Anti-CD45/RBITC 红色荧光素罗丹明标记CD45抗体 IgGMulti-class antibodies规格： 0.2ml

Rhesus antibody Rh DRD3 多巴胺受体D3抗体 规格 0.1ml

Anti-Ins 胰岛素抗体(间接法二步法) 96T

MMP-8 英文名称： 基质金属蛋白酶-8/胶原酶2抗体 0.1ml

ACEI 英文名称： 紧张素1转换酶抑制剂抗体 0.1ml

Anti-CD45/RBITC 红色荧光素罗丹明标记CD45抗体 IgGMulti-class antibodies规格： 0.2ml

THP(Human T-H glycoprotein) ELISA Kit 人TH糖蛋白Multi-class antibodies规格： 48T

Anti-P27 kip1 P27抗体/周期素依赖激酶抑制剂Multi-class antibodies规格： 0.1ml

Rhesus antibody Rh HURP/DAP5 上调蛋白抗体 规格 0.2ml

DHEA-S(Mouse Dehydroepiandrosterone sulfate)ELISA Kit 小鼠脱氢表雄酮酯 96T

Peroxiredoxin 2 英文名称： 硫氧还蛋白过氧化物酶Ⅱ/巯基抗氧化蛋白抗体 0.2ml

CCDC43 英文名称： 卷曲螺旋结构域蛋白43抗体 0.2ml

Anti-P27 kip1 P27抗体/周期素依赖激酶抑制剂Multi-class antibodies规格： 0.1ml

Voges-Proskauer(V-P)reagentbox

GlucoseAsparagineMedium

SCHAEDLER 琼脂 SCHAEDLER AGAR 250 用于厌氧菌的分离培养(Acumedia 方法)

改良MSRV培养基250g/瓶用于沙门氏菌的选择性分离鉴别，每培养基中需添加607incubationmedia改良MSRV培养基250g/瓶用于沙门氏菌的选择性分离鉴别，每培养基中需添加607

GVPC琼脂平板(9cm) 10个/包 用于军团菌的选择性分离培养

ModifiedBufferedPeptoneWater

快速试验琼脂 H2S Test Medium Agar 用于弯曲杆菌的试验(GB标准)

EMB琼脂 EMB Agar 250克 肠道致病菌的分离，亦可用于大肠埃希氏菌的分离和鉴别

Thayer-Martin选择性琼脂基础(MTMⅡ琼脂基础)250g/瓶用于致病性奈瑟氏菌的分离incubationmediaThayer-Martin选择性琼脂基础(MTMⅡ琼脂基础)250g/瓶用于致病性奈瑟氏菌的分离

EnterobacterSakazakiiChr

肌痉挛相关EPM2蛋白抗体费用 山梨醇麦康凯琼脂平板  规格：  90mmX20个  用途：  用于致病性大肠杆菌(包括O157：H7）的分离培养。

改良EC肉汤（mEC+n）  规格：  225mlX6瓶  用途：  用于致病性大肠杆菌O157：H7的增菌培养。

MUG培养基  规格：  5mlX20支  用途：  用于大肠埃希氏菌的快速测定，可观察荧光和吲哚试验。

煌绿乳糖胆盐肉汤（BGLB）  规格：  10mlX20支  用途：  用于多管发酵法测定大肠菌群的确证试验。