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: 日本网站大全 | 亚洲高清资源在线观看 | AV无码专区亚洲AV极速版 | 91精品啪在线观看国产线免费 | 亚洲AV成人综合网久久 | 国产日本在线 | 日本黄色免费在线观看 | 天天天做夜夜夜做无码 | 精品一区二区久久久久 | 欧美精品一区二区三区久久久竹菊 | 麻豆影视免费观看 | 国产高清激情在线观看 | 好硬啊进得太深了A片无码公司 | 91久久国产精品 | 国精品人妻无码一区二区三区软件 | 93久久精品日日躁夜夜躁欧美 | 最新av在线网址 | 色播影院私人影院免费 | 动漫精品无码h在线观看 | 久久精品aⅴ无码中文字字幕不卡 | 一本大道香一蕉久 | 久久国产精品免费专区 | 久久国产精品无码一区二区三区 | 亚洲国产日韩欧美视频二区 | 一区二区三区免费观看 | 弄逼视频 | 亚洲高清在线播放 | 怡春院性无码免费视频 | 999久久国产| 亚洲日韩日本中文在线 | 亚洲av无一区二区三区 | 日本美女一区二区三区插比 | 欧美性生活视频网站 | 人妻av一区二区三区精品 | 麻豆网站在线免费观看 | 日本不卡一区二区三区在线 | 97久久人人超碰超碰窝窝 | 香港aa三级久久三级 | 欧美日韩一本的免费高清视频 | 久久国产一| 四十如虎的丰满熟妇啪啪 |