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產(chǎn)品訂購(gòu)信息：
英文名稱  Anti-Nephrin 

中文名稱   腎小球細(xì)胞粘附分子受體抗體費(fèi)用 

別    名  CNF; NPHN; Nephrosis 1 congenital Finnish type; NPHS 1; NPHS1; Renal glomerulus specific cell adhesion receptor; Renal glomerulus-specific cell adhesion receptor; NPHN_HUMAN .

濃    度   1mg/1ml

規(guī) 格  0.1ml/100μg 0.2ml/200μg

抗體來(lái)源   Rabbit

克隆類型   polyclonal

交叉反應(yīng)   Human, Mouse, Rat 

產(chǎn)品類型   一抗  

研究領(lǐng)域    信號(hào)轉(zhuǎn)導(dǎo) 細(xì)胞骨架

蛋白分子量  predicted molecular weight: 138kDa

性    狀   Lyophilized or Liquid

免 疫 原 KLH conjugated synthetic peptide derived from human Nephrin N-terminus 

亞    型   IgG

純化方法   affinity purified by Protein A

儲(chǔ) 存 液   0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide

腎小球細(xì)胞粘附分子受體抗體費(fèi)用 產(chǎn)品應(yīng)用    WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:100-500 IF=1:100-500

（石蠟切片需做抗原修復(fù)） 

 not yet tested in other applications.

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

保存條件  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. 

產(chǎn)品介紹 Primary steroid resistant nephrotic syndrome (SRNS) is characterized by childhood onset of proteinuria and progression to end stage renal disease. Kidney podocytes and their slit diaphragms form the final barrier to urinary protein loss. Congenital nephrotic syndrome (CNS) is caused by mutations in NPHS1 (nephrin) or NPHS2. Nephrin, a recently identified protein is a member of a group of podocyte proteins that constitute major component of the slit diaphragm especially in the foot process. Nephrin, a cell adhesion molecule, may play a crucial role in maintaining the glomerular filtration barrier. Recent studies have suggested that mutations in the gene for Nephrin reportedly lead to congenital nephrosis. Three novel podocyte proteins, Podocin, Nephrin and alpha Actinin 4 have been identified in congenital and experimental models of proteinuria. The role of Nephrin in anti apoptotic activity in podocyte slit diaphragm is believed to be associated with vascular endothelial derived growth factors VEGF signaling.

Function : Seems to play a role in the development or function of the kidney glomerular filtration barrier. Regulates glomerular vascular permeability. May anchor the podocyte slit diaphragm to the actin cytoskeleton. Plays a role in skeletal muscle formation through regulation of myoblast fusion.Subunit : Interacts with CD2AP (via C-terminal domain). Interacts with MAGI1 (via PDZ 2 and 3 domains) forming a tripartite complex with IGSF5/JAM4. Interacts with DDN; the interaction is direct. Self-associates (via the Ig-like domains). Also interacts (via the Ig-like domains) with KIRREL/NEPH1 and KIRREL2; the interaction with KIRREL is dependent on KIRREL glycosylation. Forms a complex with ACTN4, CASK, IQGAP1, MAGI2, SPTAN1 and SPTBN1 (By similarity). Interacts with NPHS2.Subcellular Location : Cell membrane; Single-pass type I membrane protein (Potential). Note=Predominantly located at podocyte slit diaphragm between podocyte foot processes. Also associated with podocyte apical plasma membrane.Tissue Specificity : Specifically expressed in podocytes of kidney glomeruli. Expressed in kidney glomeruli. In the embryo,expressed in the mesonephric kidney at E11 with strong expression in cranial tubules with podocyte-like structures. Expression is observed in the podocytes of the developing kidney from E13. High expression is also detected in the developing cerebellum, hindbrain, spinal cord, retina and hypothalamus. Expressed in skeletal muscle during myoblast fusion such as in the adult following acute injury and in the embryo but not detected in uninjured adult skeletal muscle. Isoform 1 and isoform 2 are expressed in the newborn brain and developing cerebellum. Isoform 1 is the predominant isoform in adult kidney.Post-translational modifications : Phosphorylated at Tyr-1193 by FYN, leading to the recruitment and activation of phospholipase C-gamma-1/PLCG1.DISEASE : Defects in NPHS1 are the cause of nephrotic syndrome type 1 (NPHS1) [MIM:256300]; also known as Finnish congenital nephrosis (CNF). A renal disease characterized clinically by proteinuria, hypoalbuminemia, hyperlipidemia, and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure.Similarity : Belongs to the immunoglobulin superfamily.Contains 1 fibronectin type-III domain.Contains 8 Ig-like C2-type (immunoglobulin-like) domains.Database links : Entrez Gene: 4868 HumanEntrez Gene: 54631 MouseOmim: 602716 HumanSwissProt: O60500 HumanSwissProt: Q9QZS7 MouseUnigene: 122186 HumanUnigene: 437830 Mouse

bFGF-9 ELISA Kit 大鼠堿性成纖維細(xì)胞生長(zhǎng)因子9Multi-class antibodies規(guī)格： 48T

Anti-ApoH/FITC 熒光素標(biāo)記載脂蛋白H抗體IgGMulti-class antibodies規(guī)格： 0.2ml

Rhesus antibody Rh FAM50B FAM50B蛋白抗體 規(guī)格 0.2ml

IL-17(Human Interleukin 17) ELISA Kit 人白介素17 96T

MRPS22 英文名稱： 線粒體核糖體蛋白S22抗體 0.1ml

Aspartate Aminotransferase 英文名稱： 谷草轉(zhuǎn)酶抗體 0.2ml

Anti-ApoH/FITC 熒光素標(biāo)記載脂蛋白H抗體IgGMulti-class antibodies規(guī)格： 0.2ml

aFGF-1 ELISA Kit 大鼠酸性成纖維細(xì)胞生長(zhǎng)因子1Multi-class antibodies規(guī)格： 48T

Anti-phospho-APP(phospho Thr668) /FITC 熒光素標(biāo)記兔抗人、大、小鼠磷酸化APP淀粉樣肽前體蛋白(p-APP Thr668)抗體IgGMulti-class antibodies規(guī)格： 0.2ml

Rhesus antibody Rh FAM46D/CT112 /抗原112抗體 規(guī)格 0.2ml

IL-1(Human Interleukin 1) ELISA Kit 人白介素1 96T

MLCK 英文名稱： 肌球蛋白輕鏈激酶抗體 0.1ml

ATG4D 英文名稱： 自噬相關(guān)蛋白4D抗體 0.2ml

Anti-phospho-APP(phospho Thr668) /FITC 熒光素標(biāo)記兔抗人、大、小鼠磷酸化APP淀粉樣肽前體蛋白(p-APP Thr668)抗體IgGMulti-class antibodies規(guī)格： 0.2ml

Anti-Phospho-Connexin 43 (Ser368) /FITC 熒光素標(biāo)記兔抗人、大、小鼠等磷酸化Connexin 43蛋白抗體IgGMulti-class antibodies規(guī)格： 0.2ml

Anti-p90RSK/FITC 熒光素標(biāo)記絲氨酸/蘇氨酸激酶p90RSK蛋白抗體IgGMulti-class antibodies規(guī)格： 0.2ml

Rhesus antibody Rh CATSPER 陽(yáng)離子通道相關(guān)蛋白1抗體 規(guī)格 0.2ml

FRA2/FOSL2(Fos related/like antigen 2) FOS樣抗原2 0.5mg

Histone H3 英文名稱： 組蛋白H3抗體 0.1ml

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en80-CornAgarMedium

FB1/Half Fraser配套試劑(黃素、萘啶酮酸、檸檬酸鐵銨、氯化) 4支/套×5(g) incubation media FB1/Half Fraser配套試劑(黃素、萘啶酮酸、檸檬酸鐵銨、氯化) 4支/套×5(g)

ER培養(yǎng)基 Eriksson Medium 10升 BR

MiddleBrook7H250用于分枝桿菌的分離培養(yǎng)incubationmediaMiddleBrook7H250用于分枝桿菌的分離培養(yǎng)

NAC液體培養(yǎng)基 250g 用于綠膿假單胞菌的增菌培養(yǎng)

Indiaink

李斯特氏菌選擇富集添加劑 incubation media 李斯特氏菌選擇富集添加劑

3%氯堿性蛋白胨水 250g 用于副溶血性弧菌增菌培養(yǎng)。

Wistar胎鼠皮層神經(jīng)元完全培養(yǎng)基CorticalneuronsofWistarfetalratscompletemedium

Sabouraud’sAgar

腎小球細(xì)胞粘附分子受體抗體費(fèi)用 PH9.6肉湯  規(guī)格：  20支/盒  用途：  細(xì)菌生化鑒定

6.5%高鹽肉湯  規(guī)格：  20支/盒  用途：  細(xì)菌生化鑒定

UIM培養(yǎng)管  規(guī)格：  20支/盒  用途：  細(xì)菌生化鑒定

SIM培養(yǎng)管  規(guī)格：  20支/盒  用途：  細(xì)菌生化鑒定

抗體的生物素化標(biāo)記實(shí)驗(yàn)要點(diǎn)：

1. 腎小球細(xì)胞粘附分子受體抗體費(fèi)用 如在反應(yīng)混合液中有疊氮鈉或游離氨基存在,會(huì)抑制標(biāo)記反應(yīng)。因此,蛋白質(zhì)在反應(yīng)前要對(duì) 0.1mol/L碳酸氫鈉緩沖液或0.5mol/L硼酸緩沖液充分透析；

2.所用的NHSB及待生物素化蛋白質(zhì)之間的分子比按蛋白質(zhì)表面的ε-氨基的密度會(huì)有所不同,選擇不當(dāng)則影響標(biāo)記的效率,應(yīng)先用幾個(gè)不同的分子比來(lái)篩選最適條件；

3.用NHSB量過(guò)量也是不利的,抗原的結(jié)合位點(diǎn)可能因此被封閉,導(dǎo)致抗體失活；

4.由于抗體的氨基不易接近可能造成生物素化不足,此時(shí)可加入去污劑如 Triton x-100, Tween20等；

5.當(dāng)游離ε-氨基(賴氨酸殘基的氨基)存在于抗體的抗原結(jié)合位點(diǎn)時(shí),或位于酶的催化位點(diǎn)時(shí),生物素化會(huì)降低或損傷抗體蛋白的結(jié)合力或活性；

6.生物素還可能與不同的功能基團(tuán),如羰基、氨基、巰基、異咪唑基及苯酚基,也可與糖基共價(jià)結(jié)合；

7.交聯(lián)反應(yīng)后,應(yīng)充分透析,否則,殘余的生物素會(huì)對(duì)生物素化抗體與親和素的結(jié)合產(chǎn)生競(jìng)爭(zhēng)作用；

8.在細(xì)胞的熒光標(biāo)記實(shí)驗(yàn)中,中和親和素的本底低,但由于鏈霉親和素含有少量正電荷,故對(duì)某些細(xì)胞可導(dǎo)致高本底。

抗體的鑒定：

1）腎小球細(xì)胞粘附分子受體抗體費(fèi)用 抗體的效價(jià)鑒定：不管是用于診斷還是用于,制備抗體的目的都是要求較高效價(jià)。不同的抗原制備的抗體,要求的效價(jià)不一。鑒定效價(jià)的方法很多,包括有試管凝集反應(yīng),瓊脂擴(kuò)散試驗(yàn),酶聯(lián)免疫吸附試驗(yàn)等。常用的抗原所制備的抗體一般都有約成的鑒定效價(jià)的方法,以資比較。如制備抗抗體的效價(jià),一般就采用瓊脂擴(kuò)散試驗(yàn)來(lái)鑒定。

2）抗體的特異性鑒定：抗體的特異性是指與相應(yīng)抗原或近似抗原物質(zhì)的識(shí)別能力。抗體的特異性高,它的識(shí)別能力就強(qiáng)。衡量特異性通常以交叉反應(yīng)率來(lái)表示。交叉反應(yīng)率可用競(jìng)爭(zhēng)抑制試驗(yàn)測(cè)定。以不同濃度抗原和近似抗原分別做競(jìng)爭(zhēng)抑制曲線,計(jì)算各自的結(jié)合率,求出各自在IC50時(shí)的濃度,并按公式計(jì)算交叉反應(yīng)率。 

如果所用抗原濃度IC50濃度為pg/管,而一些近似抗原物質(zhì)的IC50濃度幾乎是無(wú)窮大時(shí),表示這一抗血清與其他抗原物質(zhì)的交叉反應(yīng)率近似為0,即該血清的特異性較好。

3）抗體親和力：是指抗體和抗原結(jié)合的牢固程度。親和力的高低是由抗原分子的大小,抗體分子的結(jié)合位點(diǎn)與抗原決定簇之間立體構(gòu)型的合適度決定的。有助于維持抗原抗體復(fù)合物穩(wěn)定的分子間力有氫鍵,疏水鍵,側(cè)鏈相反電荷基因的庫(kù)侖力,范德華力和空間斥力。親和力常以親和常數(shù)K表示,K的單位是L/mol?？贵w親和力的測(cè)定對(duì)抗體的篩選,確定抗體的用途,驗(yàn)證抗體的均一性等均有重要意義。