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
日日干狠狠操,伊人久久综合精品一区二区三区,在线观看v片
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-GCSH/BF488 Conjugated antibody (bs-13323R-BF488)
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-BF488
英文名稱 Rabbit Anti-GCSH/BF488 Conjugated antibody
中文名稱 BF488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: 99r在线| 国产精品JⅠZZ视频 av解说在线精品 | 粉嫩av一区二区三区入口 | 久久午夜影视 | 我想看一级黄色毛片 | 热99re久久国免费超精品首页 | 精品欧洲AV无码一区二区三区 | 新国产三级在线观看播放 | 欧美高清一级片 | av资源站最稳定的资源站 | 国产精品一区在线观看 | 亚洲欧美激情视频 | 日本三级全黄三级a | 国产免费av一区二区 | 免费一级成人毛片 | 亚洲AV无码AV制服丝袜在线 | 午夜影视免费 | 成人av网站在线播放 | 超碰中文在线 | 欧美亚洲性视频 | 亚洲人和日本人hd | 亚洲精品国偷拍自产在线观看蜜臀 | 中文字幕在线观看 | 91新网址 | 欧美熟妇的荡欲在线观看 | 韩国黄色精品 | 永久不封国产av毛片 | 国产精品亚洲片在线观看不卡 | 两性午夜色视频免费网站 | 亚洲一区二区二区久久成人婷婷 | 波多野结衣一区二区免费高清 | 国内av一区二区 | 国产精品99久久久久久久vr | 国产最新精品 | 日本不卡免费一区 | 欧美日韩国产网站 | 亚洲婷婷一区二区三区在线观看 | 男女插插免费视频 | 男生戳美女两腿中间那个视频 | 日韩一级免费片 | 您好星期六2024最新免费观看 |