亚洲制服欧美另类-午夜激情av电影-日本高清中文字幕一区二区三区-中国欧美日韩一区二区三区-欧洲亚洲日本韩国-成人欧美激情一区二区-亚洲偷偷自拍高清

掃碼關(guān)注公眾號           掃碼咨詢技術(shù)支持           掃碼咨詢技術(shù)服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術(shù)答疑  技術(shù)支持  質(zhì)量反饋  關(guān)于我們  聯(lián)系我們
久久99国产视频,欧洲精欧洲精品高清一区,欧美日韩第一区二区三区
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-PMP22/BF594 Conjugated antibody (bs-0235R-BF594)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-0235R-BF594
英文名稱 Rabbit Anti-PMP22/BF594 Conjugated antibody
中文名稱 BF594標記的外周髓鞘蛋白-22抗體
別    名 GAS3; CMT1A; CMT1E; DSS; GAS-3; Growth Arrest Specific 3; Growth arrest-specific protein 3; HMSNIA; HNPP; MGC20769; Peripheral Myelin Protein 22; PMP-22; PMP22; PMP22_HUMAN; Sp110; Trembler.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 免疫學  神經(jīng)生物學  糖蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse, Rat,  (predicted: Human, )
產(chǎn)品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 22kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PMP-22 C-terminus
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.
產(chǎn)品介紹 background:
PMP22 is a 22 kDa glycoprotein expressed in the compact myelin of the peripheral nervous system. In the peripheral nervous system, PMP 22 is produced by myelinating Schwann cells and is coexpressed with the genes for myelin basic protein (MBP) during nerve development and regeneration. Alterations in the level of this protein cause several genetic human diseases. If the protein is duplicated, patients develop Charcot Marie Tooth disease. If one copy of the gene is deleted, they suffer from the inherited tendency to pressure palsies.

Function:
Might be involved in growth regulation, and in myelinization in the peripheral nervous system.

Subcellular Location:
Cell membrane; Multi-pass membrane protein.

DISEASE:
Defects in PMP22 are the cause of Charcot-Marie-Tooth disease type 1A (CMT1A) [MIM:118220]; also known as hereditary motor and sensory neuropathy IA. CMT1A is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT1 group are characterized by severely reduced nerve conduction velocities (less than 38 m/sec), segmental demyelination and remyelination with onion bulb formations on nerve biopsy, slowly progressive distal muscle atrophy and weakness, absent deep tendon reflexes, and hollow feet. CMT1A inheritance is autosomal dominant.
Defects in PMP22 are a cause of Dejerine-Sottas syndrome (DSS) [MIM:145900]; also known as Dejerine-Sottas neuropathy (DSN) or hereditary motor and sensory neuropathy III (HMSN3). DSS is a severe degenerating neuropathy of the demyelinating Charcot-Marie-Tooth disease category, with onset by age 2 years. DSS is characterized by motor and sensory neuropathy with very slow nerve conduction velocities, increased cerebrospinal fluid protein concentrations, hypertrophic nerve changes, delayed age of walking as well as areflexia. There are both autosomal dominant and autosomal recessive forms of Dejerine-Sottas syndrome.
Defects in PMP22 are a cause of hereditary neuropathy with liability to pressure palsies (HNPP) [MIM:162500]; an autosomal dominant disorder characterized by transient episodes of decreased perception or peripheral nerve palsies after slight traction, compression or minor traumas.
Defects in PMP22 are the cause of Charcot-Marie-Tooth disease type 1E (CMT1E) [MIM:118300]; also known as Charcot-Marie-Tooth disease and deafness autosomal dominant. CMT1E is an autosomal dominant form of Charcot-Marie-Tooth disease characterized by the association of sensorineural hearing loss with peripheral demyelinating neuropathy.
Defects in PMP22 may be a cause of inflammatory demyelinating polyneuropathy (IDP) [MIM:139393]. IDP is a putative autoimmune disorder presenting in an acute (AIDP) or chronic form (CIDP). The acute form is also known as Guillain-Barre syndrome.

Similarity:
Belongs to the PMP-22/EMP/MP20 family.

Database links:

Entrez Gene: 5376 Human

Entrez Gene: 24660 Rat

Omim: 601097 Human

SwissProt: Q01453 Human

SwissProt: P25094 Rat

SwissProt: Q07066 Rat

Unigene: 372031 Human

Unigene: 1476 Rat



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

神經(jīng)生物學相關(guān)蛋白(Neurobiology)
外周髓鞘蛋白22(PMP22)是一種糖蛋白,在外周神經(jīng)系統(tǒng)中的致密肌纖維素中表達。它由髓鞘雪旺氏細胞產(chǎn)生,并在神經(jīng)發(fā)育和再生過程中與MBP和Po蛋白共同表達。該蛋白表達水平變化可引起幾種人類遺傳性疾病,如果該蛋白增多,則病人會發(fā)生Charcot-Marie-Tooth疾病,如果缺失,則易患壓力麻痹的遺傳傾向。
版權(quán)所有 2004-2026 www.a6308.cn 北京博奧森生物技術(shù)有限公司
通過國際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫(yī)療器械-質(zhì)量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網(wǎng)安備110107000727號
欧美A级毛欧美1级a大片式放 | 亚洲精品1卡2卡三卡23卡 | 成人国产精品日本在线 | 日韩超碰人人爽人人做人人天 | 亚洲AVAV天堂AV在线网爱情 | 飘雪影视在线观看免费观看西瓜 | 色噜噜人妻丝袜aV先锋影音先 | 精品久久久久久久一区二区 | 后入骚妇内射AV | 美女又大又黄www免费网站 | 伊人狠狠丁香婷婷综合尤物 | 99久久久精品综合网 | 亚洲成人在线免费观看 | 激情内射人妻1区2区3区 | 久久中文字幕亚洲一区二区 | 四虎影视国产精品亚洲精品 | 粗大的内捧猛烈进出视频网 | 国产一级a毛一级a看免费视频 | 精品国产鲁一鲁一区二区66AV | 欧美人与拘牲交大全O人禾 天天躁日日躁狠狠躁日日躁黑人 | 国产无套内射又大又猛又粗又爽 | 久久综合九色综合91 | 欧美片内射欧美美美妇 | 亚洲中文字幕永久在线不卡 | 欧美日韩不卡高清在线看 | 欧美成人免费观看久久 | 国产精品xxx电影 | 小宝极品内射国产在线 | 嗯好湿用力的啊c进来动态图 | 亚洲精品嫩草研究院久久 | 国产精品扒开腿做爽爽爽视频 | 日本VS亚洲VS韩国一区三区四区 | 最近免费中文字幕大全免费版视频 | 久久综合九色综合91 | 久久国产V一级毛多内射 | 国产在线精品亚洲另类 | 91精品一区二区三区在线观看 | 国产精品自慰在线观看 | 在线看片免费人成视频电影sM | 4399视频免费观看大全 | 美女裸胸不打马赛克 |