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
久久久www视频,久久久久久久久中文字幕,182tv人之初午夜精品视频
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-GCSH/BF647 Conjugated antibody (bs-13323R-BF647)
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-BF647
英文名稱 Rabbit Anti-GCSH/BF647 Conjugated antibody
中文名稱 BF647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: 久久伊人色AV天堂九九小黄鸭 | 在线观看热码亚洲av每日更新 | 精品久久久无码中文字幕 | 91精品国产一区二区三区 | 成人AV导航 | 999ZYZ玖玖资源站永久 | 国产亚洲精选美女久久久久 | 亚洲一区二区三区尿失禁 | 欧美6一10娇小xxxx | 丰满少妇被猛烈进入毛片 | 免费一对一刺激互动聊天软件 | 精品欧美在线观看 | 91播放在线 | 成人性生交大片免费 | 日本又色又爽又黄的a片中文字幕 | 亚洲AV人无码综合在线观看 | 黄网站免费久久 | 久久欧美高清二区三区 | 免费播放毛片 | 久久综合中文字幕一区二区三区 | 91在现看| 久久之久| 国产卡一卡二卡乱码 | 久久嫩草 | 日韩国产中文字幕 | 国产亚洲精品AA片在线播放网站 | jiujiure精品视频播放 | 成人播放 | 国产色综合一区二区三区 | 日韩一区二区在线观看 | 天下免费大全正版资料 | 日韩黄色高清 | 色99热久久偷拍 | 久久毛片免费观看 | 69精品久久久久9999不卡片 | 国产真实迷奷系列 | 四虎黄色影院 | 日韩久久无码免费毛片软件 | 怡春院免费视频 | 亚洲激情成人网 | 性久久久久久 |