吃奶呻吟打开双腿做受动态图 -亚洲色偷偷色噜噜狠狠99网-日韩精品极品视频在线观看免费-来一水AV@lysav

掃碼關(guān)注公眾號(hào)           掃碼咨詢技術(shù)支持           掃碼咨詢技術(shù)服務(wù)
  
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  人才招聘  關(guān)于我們  聯(lián)系我們
后入内射无码人妻一区,欧美精产国品一二三产品区别在哪
Rabbit Anti-Gigaxonin/Cy7 Conjugated antibody (bs-11025R-Cy7)
訂購(gòu)熱線:400-901-9800
訂購(gòu)郵箱:sales@xucheq.com
訂購(gòu)QQ:  400-901-9800
技術(shù)支持:techsupport@xucheq.com
說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-11025R-Cy7
英文名稱1 Rabbit Anti-Gigaxonin/Cy7 Conjugated antibody
中文名稱 Cy7標(biāo)記的巨軸索神經(jīng)病蛋白GAN抗體
別    名 FLJ38059; GAN; GAN1; Kelch-like protein 16; giant axonal neuropathy; KLHL16; GAN_HUMAN.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞粘附分子  細(xì)胞骨架  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 68kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Gigaxonin
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 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:
Gigaxonin, also refered to as giant axonal neuropathy, GAN1, or KLHL16, controls protein degradation and is essential for neuronal function and survival. Gigaxonin is a member of the cytoskeletal BTB/kelch repeat family and influences cytoskeletal organization and dynamics, playing a large role in neurofilament architecture. The amino terminal BTB domain of gigaxonin binds to the ubiquitin-activating enzyme E1, while the carboxy-terminal kelch repeat domain interacts directly with the light chain of microtubule-associated protein 1B (MAP1B), and tags it for degredation. Overexpression of MAP1B may lead to neuronal cell death, whereas a reduction of MAP1B significantly improves the survival rate of neurons. Mutations in the Gigaxonin gene result in human giant axonal neuropathy (GAN), an autosomal recessive neurodegenerative disorder characterized by axonal degeneration caused by cytoskeletal abnormalities, including accumulated intermediate filaments.

Function:
Mutations in gigaxonin result in a sensory and motor neuropathy called Giant Axonal Neuropathy (GAN). Giant axonal neuropathy, a severe autosomal recessive sensorineural neuropathy affecting both the peripheral nerves and the central nervous system, is characterized by neurofilament accumulation, leading to segmental distention of axons. Gigaxonin is a member of the cytoskeletal BTB/kelch (Broad-Complex, Tramtrack and Bric a brac) repeat family. Gigaxonin contains an N-terminal BTB domain followed by 6 kelch repeats, which were predicted to adopt a beta-propeller shape. Gigaxonin controls protein degradation and is essential for neuronal function and survival. Substrate-specific adapter of an E3 ubiquitin-protein ligase complex which mediates the ubiquitination and subsequent proteasomal degradation of target proteins. Controls degradation of TBCB. Controls degradation of MAP1B and MAP1S, and is critical for neuronal maintenance and survival

Subunit:
Interacts with TBCB. Interacts with CUL3. Part of a complex that contains CUL3, RBX1 and GAN. Interacts (via BTB domain) with UBA1. Interacts (via Kelch domains) with MAP1B (via C-terminus) and MAP1S (via C-terminus).

Subcellular Location:
Cytoplasmic; Cytoskeleton.

Tissue Specificity:
Expressed in brain, heart and muscle.

Post-translational modifications:
Ubiquitinated by E3 ubiquitin ligase complex formed by CUL3 and RBX1 and probably targeted for proteasome-independent degradation.

DISEASE:
Defects in GAN are the cause of giant axonal neuropathy (GAN) [MIM:256850]. GAN is a severe autosomal recessive sensorimotor neuropathy affecting both the peripheral nerves and the central nervous system. It is characterized by neurofilament accumulation, leading to segmental distention of axons.

Similarity:
Contains 1 BACK (BTB/Kelch associated) domain.
Contains 1 BTB (POZ) domain.
Contains 6 Kelch repeats.

Database links:

Entrez Gene: 8139 Human

Entrez Gene: 209239 Mouse

Omim: 605379 Human

SwissProt: Q9H2C0 Human

SwissProt: Q8CA72 Mouse

Unigene: 112569 Human

Unigene: 132992 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權(quán)所有 2004-2026 www.xucheq.com 北京博奧森生物技術(shù)有限公司
通過(guò)國(guó)際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書(shū)編號(hào): 00124Q34771R2M/1100
通過(guò)國(guó)際醫(yī)療器械-質(zhì)量管理體系ISO 13485:2016 GB/T 42061-2022    證書(shū)編號(hào): CQC24QY10047R0M/1100
京ICP備05066980號(hào)-1         京公網(wǎng)安備110107000727號(hào)
成av人片一区二区三区久久| 处破痛哭A√18成年片免费| 偷窥学校女厕撒尿BBBBB| 国产精品人人妻人色五月| 性欧美大战久久久久久久| 黑人巨大精品欧美一区二区免费| 无遮掩60分钟从头啪到尾| 无码人妻一区二区三区免费N鬼沢 久久精品色妇熟妇丰满人妻5O | 国产精品无码一区二区三区| 天堂网在线最新版WWW中文网| 国产特黄级AAAAA片免| 狠狠色噜噜狠狠狠狠AV不卡| 精品夜夜澡人妻无码AV蜜桃| 丰满少妇a级毛片| 精品国产制服丝袜高跟| 男女做爰猛烈叫床视频动态图| 日韩AV高清在线观看| 欧美精品一区二区少妇免费A片 | АⅤ资源天堂资源库在线| 欧美三级A做爰在线观看| 亚洲午夜无码久久久久| 国色天香久久久久久久小说| YYY6080韩国三级理论| 国产成人精品免高潮在线观看| 精品人妻大屁股白浆无码| 隔着内裤揉搓她的花蒂H漫画| 亚洲精品久久久久久| 中文字幕AV一区二区三区| 久久高清内射无套| 国产成人久久精品激情| 久久影院午夜理论片无码| 亚洲AV成人无码网天堂| 久久免费看少妇高潮A片| AV网站在线观看| 精品欧美一区二区三区久久久| 日本熟妇色XXXXX日本免费看 | 亚洲av无码乱码在线观看性色| 少妇人妻系列1~100| 成人毛片18女人毛片免费看| 小处雏一区二区三区精品视频| 国产乱人伦偷精品视频免下载|