NGLY1 polyclonal antibody
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More Files
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Immunohistochemical staining of human stomach with NGLY1 polyclonal antibody (Cat # PAB23068) shows strong cytoplasmic positivity, with a granular pattern in glandular cells.
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Specification
Product Description
Rabbit polyclonal antibody raised against recombinant NGLY1.
Immunogen
Recombinant protein corresponding to amino acids of human NGLY1.
Sequence
ISDEDFLLLELLHWFKEEFFHWVNNVLCSKCGGQTRSRDRSLLPSDDELKWGAKEVEDHYCDACQFSNRFPRYNNPEKLLETRCGR
Host
Rabbit
Reactivity
Human
Form
Liquid
Purification
Antigen affinity purification
Isotype
IgG
Recommend Usage
Immunohistochemistry (1:20-1:50)
The optimal working dilution should be determined by the end user.Storage Buffer
In PBS, pH 7.2 (40% glycerol, 0.02% sodium azide)
Storage Instruction
Store at 4°C. For long term storage store at -20°C.
Aliquot to avoid repeated freezing and thawing.Note
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Applications
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Immunohistochemical staining of human stomach with NGLY1 polyclonal antibody (Cat # PAB23068) shows strong cytoplasmic positivity, with a granular pattern in glandular cells. -
Gene Info — NGLY1
Entrez GeneID
55768Protein Accession#
Q96IV0Gene Name
NGLY1
Gene Alias
FLJ11005, FLJ12409, PNG1
Gene Description
N-glycanase 1
Omim ID
610661Gene Ontology
HyperlinkGene Summary
This gene encodes an enzyme that catalyzes hydrolysis of an N(4)-(acetyl-beta-D-glucosaminyl) asparagine residue to N-acetyl-beta-D-glucosaminylamine and a peptide containing an aspartate residue. The encoded enzyme may play a role in the proteasome-mediated degradation of misfolded glycoproteins. Multiple transcript variants encoding different isoforms have been found for this gene
Other Designations
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Interactome
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Disease
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Publication Reference
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Variants in NGLY1 lead to intellectual disability, myoclonus epilepsy, sensorimotor axonal polyneuropathy and mitochondrial dysfunction.
Panneman DM, Wortmann SB, Haaxma CA, van Hasselt PM, Wolf NI, Hendriks Y, Küsters B, van Emst-de Vries S, van de Westerlo E, Koopman WJH, Wintjes L, van den Brandt F, de Vries M, Lefeber DJ, Smeitink JAM, Rodenburg RJ.
Clinical Genetics 2020 Apr; 97(4):556.
Application：WB-Ce, WB-Ti, Human, Fibroblast homogenates, Muscle homogenates.
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Variants in NGLY1 lead to intellectual disability, myoclonus epilepsy, sensorimotor axonal polyneuropathy and mitochondrial dysfunction.
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