亚洲一区二区三区看片,日韩欧美九九九久久久,精品国产日韩亚洲一区在线,久久噜噜噜久久熟女精品

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
精品人妻少妇一区二区三区,欧美一区二区三区热
首頁(yè) > 產(chǎn)品中心 > 一抗 > 產(chǎn)品信息
Rabbit Anti-AFG3L2  antibody (bs-11704R)  
~~~促銷代碼KT202411~~~
訂購(gòu)熱線:400-901-9800
訂購(gòu)郵箱:sales@www.lalhoau.cn
訂購(gòu)QQ:  400-901-9800
技術(shù)支持:techsupport@www.lalhoau.cn
說(shuō)明書(shū): 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包裝/詢價(jià)

產(chǎn)品編號(hào) bs-11704R
英文名稱 Rabbit Anti-AFG3L2  antibody
中文名稱 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 .  
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) Mouse,Rat (predicted: Human,Pig,Sheep,Cow,Chicken,Dog,Horse)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 89kDa
細(xì)胞定位 細(xì)胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human AFG3L2: 531-600/797 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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.

SWISS:
Q9Y4W6

Gene ID:
10939

Database links:

Entrez Gene: 10939 Human

Omim: 604581 Human

SwissProt: Q9Y4W6 Human

Unigene: 726355 Human



產(chǎn)品圖片
Paraformaldehyde-fixed, paraffin embedded (mouse cerebellum); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (AFG3L2) Polyclonal Antibody, Unconjugated (bs-11704R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (AFG3L2) Polyclonal Antibody, Unconjugated (bs-11704R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
版權(quán)所有 2004-2026 www.www.lalhoau.cn 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)
五常市| 望都县| 沙洋县| 新干县| 聊城市| 蒙阴县| 正阳县| 日喀则市| 铁岭市| 应用必备| 南安市| 丹棱县| 隆化县| 德格县| 香港| 甘孜县| 远安县| 兴仁县| 罗定市| 信阳市| 温宿县| 泽库县| 樟树市| 梁山县| 道真| 郎溪县| 永年县| 罗甸县| 高青县| 安仁县| 乌鲁木齐县| 鄄城县| 遵化市| 迭部县| 台中市| 桐城市| 新田县| 大化| 温泉县| 酉阳| 左云县|