超碰97自拍_久久99久久98精品免观看软件_在线亚洲一区_九九视频这里只有精品_国产精品一区二区欧美视频_精品国产区一区

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
黑人黄色片,国产精彩视频一区,亚洲AV丝袜美腿无码播放器
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-GCSH/PE-Cy7 Conjugated antibody (bs-13323R-PE-Cy7)
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-13323R-PE-Cy7
英文名稱 Rabbit Anti-GCSH/PE-Cy7 Conjugated antibody
中文名稱 PE-Cy7標記的甘氨酸裂解系統H蛋白抗體
別    名 GCE; GCSH; GCSH_HUMAN; Glycine cleavage system H protein; Glycine cleavage system H protein mitochondrial; Glycine cleavage system protein H (aminomethyl carrier); Glycine cleavage system protein H; Lipoic acid containing protein; mitochondrial; Mitochondrial glycine cleavage system H protein; NKH.  
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  信號轉導  細胞類型標志物  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Horse, )
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 14kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GCSH
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:
GCSH is a 173 amino acid mitochondrial protein that contains one lipoyl-binding domain and belongs to the gcvH family. Defects in the gene encoding GCSH are the cause of glycine encephalopathy (GCE), an autosomal recessive disease that is also referred to as non-ketotic hyperglycinemia (NKH). Characterized by severe neurological symptoms, patients with GCE have a large amount of glycine accumulated in their body fluids. The gene encoding GCSH maps to human chromosome 16, which encodes over 900 genes and comprises nearly 3% of the human genome.

Function:
The glycine cleavage system catalyzes the degradation of glycine. The H protein shuttles the methylamine group of glycine from the P protein to the T protein.

Subcellular Location:
Mitochondrion.

DISEASE:
Defects in GCSH are a cause of non-ketotic hyperglycinemia (NKH) [MIM:605899]; also known as glycine encephalopathy (GCE). NKH is an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms.

Similarity:
Belongs to the gcvH family.
Contains 1 lipoyl-binding domain.

Database links:
UniProtKB/Swiss-Prot: P23434.2

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.ggggww.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: 丰满少妇三级全黄 | 精品国产91亚洲一区二区三区婷婷 | 国产亚洲亚洲高清视频 | 色综合天天综合高清 | 你是我的女人6免费观看 | 美女张开腿让男生桶视频 | 天天操天天射综合网 | 亚洲精品久久AV无码蜜桃第1集 | 黄色工厂这里只有精品 | 精品人妻无码一区二区三区手机板 | 天堂va蜜桃| 欧美成人精品第一区 | 天天色成人网 | 欧洲尺码日本尺码专线不卡顿 | 成人免费毛片立即播放 | 亚洲第一极品精品无码 | 久久久久人妻综合免费无码 | 欧洲成人一区二区 | 亚洲一二三精品 | 免费一区二区三区四区 | 国产成人精品一区二区免费看京 | 欧美日韩中字 | 日韩免费视频一区二区 | 国产一级免费视频 | 国产精品一品二区三区四区18 | 奇色影视| 欧美顶级METART裸体全部自慰 | 久久超碰极品视觉盛宴 | hd国产人妖ts另类视频 | 美女黄色在线 | 成人深夜在线 | 亚洲一一在线 | 台湾妹中文娱乐网 | 欧美无毛视频 | 香蕉成人影院 | 久久免费视频在线观看 | 精品无码人妻一区二区三区不卡 | 81精品国产乱码久久久久久 | 亚洲成人国产综合 | 无码毛片视频一区二区三区 | 国产中文字幕乱人伦在线观看 |