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
天堂资源最新在线,天美传媒免费观看一二三在线 ,国产精品99久久久久久猫咪
Rabbit Anti-AFG3L2/Cy5 Conjugated antibody (bs-11704R-Cy5)
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: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-11704R-Cy5
英文名稱1 Rabbit Anti-AFG3L2/Cy5 Conjugated antibody
中文名稱 Cy5標(biāo)記的AFG3樣蛋白2/脊髓小腦共濟(jì)失調(diào)蛋白28抗體
別    名 SCA28; AFG3 (ATPase family gene 3, yeast) like 2; AFG3 ATPase family gene 3 like 2 (yeast); AFG3 ATPase family gene 3 like 2; AFG3 like protein 2; ATPase family gene 3 like 2; ATPase family gene 3 yeast; FLJ25993; Paraplegin like protein; SCA28; Spinocerebellar ataxia 28; AFG32_HUMAN .  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  泛素  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 89kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human AFG3L2 (531-600aa)
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:
AFG3L2 is a multi-pass membrane metalloprotease that contains one AAA (ATPase associated with diverse cellular activities) domain, a zinc-dependent binding motif, an RNA-binding region and an ATP/GTP binding site. Localizing to the mitochondrial membrane, AFG3L2 is ubiquitously expressed with highest expression levels in skeletal muscle and heart. AFG3L2 shares 69% similarity with the yeast Afg3 protein and 49% similarity with Paraplegin, a protein of mitochondria that is thought to be involved in signal transduction and chaperone-like activities. In mitochondria, AFG3L2 forms a complex with Paraplegin that is believed to regulate essential protein quality control. Mutations in the gene encoding either one of these proteins can result in hereditary spastic paraplegia, a degenerative spinal cord disorder that is characterized by muscle spasms, stiffness in the legs and, in some cases, incontinence.

Function:
AFG3L2 is a protein localized in mitochondria and closely related to paraplegin. The paraplegin gene is responsible for an autosomal recessive form of hereditary spastic paraplegia. AFG3L2 is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders and is a putative ATP dependent protease

Subunit:
Homooligomer. Interacts with SPG7; the interaction is required for the efficient assembly of mitochondrial complex I.

Subcellular Location:
Mitochondrial membrane; multipass membrane protein

Tissue Specificity:
Ubiquitous. Highly expressed in the cerebellar Purkinje cells.

DISEASE:
Defects in AFG3L2 are the cause of spinocerebellar ataxia type 28 (SCA28) [MIM:610246]. It is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA28 is an autosomal dominant cerebellar ataxia (ADCA) with a slow progressive course and no evidence of sensory involvement or cognitive impairment.
Defects in AFG3L2 are the cause of spastic ataxia autosomal recessive type 5 (SPAX5) [MIM:614487]. A neurodegenerative disorder characterized by early onset spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy.

Similarity:
In the N-terminal section; belongs to the AAA ATPase family.
In the C-terminal section; belongs to the peptidase M41 family.

Database links:

Entrez Gene: 10939 Human

Omim: 604581 Human

SwissProt: Q9Y4W6 Human

Unigene: 726355 Human



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    證書編號(hào): CQC24QY10047R0M/1100
京ICP備05066980號(hào)-1         京公網(wǎng)安備110107000727號(hào)
国产丰满老熟妇乱XXX1区| 粗大挺进朋友人妻身体里国产电影| 荫蒂添的好舒服视频囗交| 色橹橹欧美在线观看视频高清| free性熟女妓女tube| 国产精品麻豆成人AV电影艾秋| 久久泄欲网| 娇妻被领导粗又大又硬| 人体内射精一区二区三区| 国产成人精品一区二三区在线观看| 边摸边脱吃奶边高潮视频免费| 亚洲AV日韩AV永久无码色欲| 蜜臀AV国产精品久久久久| 亚洲毛片| 女厕厕露P撒尿八个少妇| 人妻VA精品VA欧美VA| 日韩精品一区二区三区在线观看| 十九岁日本电影免费完整版观看| 亚洲av午夜精品一区二区三区| 欧美性猛交XXXX乱大交3 | 欧美XXXX色视频在线观看| 无人在线观看完整免费版视频| 久久99热狠狠色AV蜜臀| 久久夜色精品国产噜噜亚洲AV | 9孩岁女精品╳片| 51国偷自产一区二区三区| 久久精品国产亚洲AV无码偷窥| 国产一国产看免费高清片| 国产成人一区二区三区| 亚洲AV无码一区二区一二区| 60欧美老妇做爰视频| 妓女精品国产噜噜亚洲AV| 无码人妻久久一区二区三区蜜桃| 小视频在线观看| 影音先锋资源站| FREE性玩弄少妇HD| 女人18毛片a级毛片免费视频| 男女多P混交群体交乱| 亚洲欧美一区二区三区在线| 人人妻人人澡人人爽精品日本| 免费A级毛片在线播放不收费|