HPA036825
Anti-NGLY1 antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
同義詞:
Ngly1 Antibody, Ngly1 Antibody - Anti-NGLY1 antibody produced in rabbit, Anti-FLJ11005, Anti-N-glycanase 1, Anti-PNG1
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About This Item
推薦產品
生物源
rabbit
共軛
unconjugated
抗體表格
affinity isolated antibody
抗體產品種類
primary antibodies
無性繁殖
polyclonal
產品線
Prestige Antibodies® Powered by Atlas Antibodies
形狀
buffered aqueous glycerol solution
物種活性
human
技術
immunohistochemistry: 1:50-1:200
免疫原序列
ISDEDFLLLELLHWFKEEFFHWVNNVLCSKCGGQTRSRDRSLLPSDDELKWGAKEVEDHYCDACQFSNRFPRYNNPEKLLETRCGR
UniProt登錄號
運輸包裝
wet ice
儲存溫度
−20°C
目標翻譯後修改
unmodified
基因資訊
human ... NGLY1(55768)
一般說明
N-glycanase 1 (NGLY1) is a cytoplasmic peptide mapped to human chromosome 3p24.2.
免疫原
N-glycanase 1 recombinant protein epitope signature tag (PrEST)
應用
All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
Anti-NGLY1 antibody produced in rabbit has been used in immunoblotting.
生化／生理作用
N-glycanase 1 (NGLY1) or peptide: N-glycanase acts on N-linked glycoproteins and glycopeptides and deglycosylates them, by cleaving on the β-aspartyl glycosylamine bond. It takes part in the endoplasmic reticulum-associated degradation (ERAD) machinery. Mutation in the NGLY1 gene leading to its deficiency, directly disturbs the endoplasmic reticulum-associated degradation pathway. It is also implicated in abnormal tear production, neurological dysfunction and liver disease. Also, NGLY1 mutation is regarded as a congenital disorder and contributes to the developmental delay and hypotonia.
特點和優勢
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
聯結
Corresponding Antigen APREST79406
外觀
Solution in phosphate buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide.
法律資訊
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
免責聲明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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儲存類別代碼
10 - Combustible liquids
水污染物質分類（WGK）
WGK 1
閃點（°F）
Not applicable
閃點（°C）
Not applicable
分析證明 (COA)
輸入產品批次/批號來搜索 分析證明 (COA)。在產品’s標籤上找到批次和批號，寫有 ‘Lot’或‘Batch’.。
Ping He et al.
Glycobiology, 25(8), 836-844 (2015-04-23)
N-Glycanase 1, encoded by NGLY1, catalyzes the deglycosylation of misfolded N-linked glycoproteins retrotranslocated into the cytosol. We identified nine cases with mutations in NGLY1. The patients show developmental delay, seizures, peripheral neuropathy, abnormal liver function and alacrima (absence of tears).
Loss of N-Glycanase 1 Alters Transcriptional and Translational Regulation in K562 Cell Lines
Mueller WF, et al.
G3: Genes, Genomes, Genetics, 10(5), 1585-1597 (2020)
A congenital disorder of deglycosylation: Biochemical characterization of N-glycanase 1 deficiency in patient fibroblasts
He P, et al.
Glycobiology, 25(8), 836-844 (2015)
Biological plasticity rescues target activity in CRISPR knock outs
Smits AH, et al.
Nature Methods, 16(11), 1087-1093 (2019)
Ivan Pavlinov et al.
Stem cell research, 56, 102554-102554 (2021-10-08)
NGLY1 deficiency is a rare recessive genetic disease caused by mutations in the NGLY1 gene which codes for N-glycanase 1 (NGLY1). Here, we report the generation of two gene corrected iPSC lines using a patient-derived iPSC line (NCATS-CL6103) that carried
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