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Key Documents

HPA036825

Sigma-Aldrich

Anti-NGLY1 antibody produced in rabbit

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

Sinônimo(s):

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

Código UNSPSC:
12352203
Número do Atlas de Proteínas Humanas:
NACRES:
NA.41

fonte biológica

rabbit

conjugado

unconjugated

forma do anticorpo

affinity isolated antibody

tipo de produto de anticorpo

primary antibodies

clone

polyclonal

linha de produto

Prestige Antibodies® Powered by Atlas Antibodies

forma

buffered aqueous glycerol solution

reatividade de espécies

human

técnica(s)

immunohistochemistry: 1:50-1:200

sequência de imunogênio

ISDEDFLLLELLHWFKEEFFHWVNNVLCSKCGGQTRSRDRSLLPSDDELKWGAKEVEDHYCDACQFSNRFPRYNNPEKLLETRCGR

nº de adesão UniProt

Condições de expedição

wet ice

temperatura de armazenamento

−20°C

modificação pós-traducional do alvo

unmodified

Informações sobre genes

human ... NGLY1(55768)

Descrição geral

N-glycanase 1 (NGLY1) is a cytoplasmic peptide mapped to human chromosome 3p24.2.

Imunogênio

N-glycanase 1 recombinant protein epitope signature tag (PrEST)

Aplicação

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.
Anti-NGLY1 antibody produced in rabbit has been used in immunoblotting.

Ações bioquímicas/fisiológicas

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.

Características e benefícios

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:
  • 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.

Ligação

Corresponding Antigen APREST79406

forma física

Solution in phosphate buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide.

Informações legais

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

Exoneração de responsabilidade

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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Código de classe de armazenamento

10 - Combustible liquids

Classe de risco de água (WGK)

WGK 1

Ponto de fulgor (°F)

Not applicable

Ponto de fulgor (°C)

Not applicable


Certificados de análise (COA)

Busque Certificados de análise (COA) digitando o Número do Lote do produto. Os números de lote e remessa podem ser encontrados no rótulo de um produto após a palavra “Lot” ou “Batch”.

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Visite a Biblioteca de Documentos

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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