97久久久精品综合88久久_亚洲国产精品一_久热热国产久热_97操操操_北条麻妃在线免费观看_精品国自产拍天天拍

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  關于我們  聯系我們
91日韩v欧美v国产v,亚洲欧美综合一区二区在线,国产亚洲婷婷
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-ABCA12/RBITC Conjugated antibody (bs-11906R-RBITC)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-11906R-RBITC
英文名稱 Rabbit Anti-ABCA12/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標記的腺苷三磷酸結合盒轉運體12抗體
別    名 ABC transporter A family member 12; ABC transporter ABCA.12; ABC12; ABCA12; ABCAC_HUMAN; AtABCA12; ATH16; ATP binding cassette 12; ATP binding cassette sub family A (ABC1) member 12; ATP binding cassette sub family A member 12; ATP binding cassette transporter 12; ATP-binding cassette 12; ATP-binding cassette sub-family A member 12; ATP-binding cassette transporter 12; Ichthyosis congenita II lamellar ichthyosis B; ICR2B; LI2; Putative ABC2 homolog 16; ABCAC_HUMAN.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  免疫學  信號轉導  新陳代謝  細胞膜蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Rabbit, )
產品應用 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 293kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ABCA12
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.
產品介紹 background:
The ATP-binding cassette (ABC) transporters, or traffic ATPases, constitute an expansive family of proteins accountable for the transport of a wide variety of substrates across cell membranes in both prokaryotic and eukaryotic cells. They also aid in the regulation of lipid transport and membrane trafficking. ABCA12 (ATP-Binding Cassette, Subfamily A, Member 12) contains two transmembrane (TM) domains, each with six membrane-spanning segments, and two nucleotide-binding domains (NBDs), which are located in the cytoplasm. ABCA12 is expressed in normal human keratinocytes (RT-PCR reveals expression in placenta, testis, fetal brain, and skin) and is upregulated during keratinization. Immunoelectron microscopy reveals that the ABCA12 protein is located in lamellar granules in the upper epidermal keratinocytes of human skin. The ABCA12 gene, which synthesizes a 2,595-amino acid protein, may produce an alternative splice variant with an in-frame deletion leading to truncation of 79 amino acids.

Function:
Probable transporter involved in lipid homeostasis.

Subcellular Location:
Membrane.

Tissue Specificity:
Mainly expressed in the stomach, placenta, testis and fetal brain.

DISEASE:
Defects in ABCA12 are the cause of ichthyosis harlequin (HI) [MIM:242500]; also known as harlequin fetus. HI is a very severe skin disorder in which the neonate is born with a thick covering of armor-like scales. The skin dries out to form hard diamond-shaped plaques separated by fissures, resembling 'armor plating'. The normal facial features are severely affected, with distortion of the lips (eclabion), eyelids (ectropion), ears, and nostrils. Affected babies are often born prematurely and rarely survive the perinatal period.
Defects in ABCA12 are the cause of ichthyosis lamellar type 2 (LI2) [MIM:601277]; also known as ichthyosis congenita IIB (ICR2B). LI is a non-bullous ichthyosis, a skin disorder characterized by abnormal cornification of the epidermis. It is one the most severe forms of ichthyoses apparent at birth and persisting throughout life. LI patients are born encased in a tight, shiny, translucent covering called collodion membrane. Over the first weeks of life, the collodion membrane is gradually replaced by generalized large, dark brown, plate-like scales with minimal to no erythroderma. Tautness of facial skin commonly results in ectropion, eclabium and scarring alopecia of the scalp. Common complications are severe heat intolerance and recurrent ear infections.

Similarity:
Belongs to the ABC transporter superfamily. ABCA family.
Contains 2 ABC transporter domains.

Database links:
UniProtKB/Swiss-Prot: Q86UK0.3

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權所有 2004-2026 www.kastlife.cn 北京博奧森生物技術有限公司
通過國際質量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫療器械-質量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網安備110107000727號
主站蜘蛛池模板: 国产乱码精品一区二区三区忘忧草 | 久久久激情综合 | 精品国产乱码91久久久久久网站 | www.狠狠| 日本孕妇潮喷高潮视频 | 久久精品国产亚洲av麻豆小说 | 理论片中文字幕 | 国产精品国产三级国AV | 99re8这里有精品热视频8在线 | 亚洲男人天堂久久 | 成人国产亚洲精品A区天堂 欧美四虎影院 | 又大又硬又爽免费视频 | 成人A片产无码免费奶头 | www国产成人免费观看视频深夜成人网 | 日韩入口 | 国产精品久久久久久婷婷动漫 | 性刺激的大陆三级视频 | 午夜小视频在线观看 | 激烈18禁高潮视频免费 | 日本黄色一级带 | av第一福利网站 | 欧美色交 | 久久天天躁狠狠躁夜夜96流白浆 | 久久久久久无码精品无码国产人妻丝瓜 | 日韩精美视频 | 在线播放视频一区 | 丰满妇女做a级毛片免费观看 | 亚洲欧洲偷自拍图片区 | 在线看免费av | 131mm亚洲国产精品久久 | 99久RE热视频这里只有精品6 | 九一免费在线观看 | 的九一视频入口在线观看 | 99毛片 | 日本草逼 | 黄色一区二区在线 | 亚洲色欲色欲综合网站 | 久久99视频这里只有精品 | 免费在线不卡视频 | 永久免费黄色 | 精品欧美亚洲韩国日本久久 |