Recombinant Anti-GNS antibody [EPR8329(2)] (ab154177)
- Datasheet
- References
- Protocols
Overview
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Product name
Anti-GNS antibody [EPR8329(2)]
See all GNS primary antibodies -
Description
Rabbit monoclonal [EPR8329(2)] to GNS -
Host species
Rabbit -
Tested applications
Suitable for: WB, IPmore details
Unsuitable for: Flow Cyt,ICC/IF or IHC-P -
Species reactivity
Reacts with: Human -
Immunogen
Synthetic peptide, corresponding to residues in Human GNS (UniProt: P15586).
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Positive control
- PC3, NCI-H460 and 293T cell lysates.
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General notes
Mouse, Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information.
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.
This product is a recombinant rabbit monoclonal antibody.
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at -20ºC. -
Storage buffer
Preservative: 0.01% Sodium azide
Constituents: 9% PBS, 40% Glycerol, 0.05% BSA, 50% Tissue culture supernatant -
Purity
Tissue culture supernatant -
Clonality
Monoclonal -
Clone number
EPR8329(2) -
Isotype
IgG -
Research areas
Associated products
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Alternative Versions
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Isotype control
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Positive Controls
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Recombinant Protein
Applications
Our Abpromise guarantee covers the use of ab154177 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
| Application | Abreviews | Notes |
|---|---|---|
| WB | 1/10000 - 1/50000. Predicted molecular weight: 62 kDa. | |
| IP | 1/10 - 1/100. |
Target
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Involvement in disease
Defects in GNS are the cause of mucopolysaccharidosis type 3D (MPS3D) [MIM:252940]; also known as Sanfilippo D syndrome. MPS3D is a form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. -
Sequence similarities
Belongs to the sulfatase family. -
Post-translational
modificationsThe form A (78 kDa) is processed by internal peptidase cleavage to a 32 kDa N-terminal species (form B) and a 48 kDa C-terminal species.
The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity. -
Cellular localization
Lysosome. - Information by UniProt
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Database links
- Entrez Gene: 2799 Human
- Omim: 607664 Human
- SwissProt: P15586 Human
- Unigene: 334534 Human
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Alternative names
- 2610016K11Rik antibody
- AU042285 antibody
- C87209 antibody
see all
Images
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![Western blot - Anti-GNS antibody [EPR8329(2)] (ab154177)](https://www.abcam.com/ps/products/154/ab154177/Images/ab154177-160109-anti-gns-antibody-epr83292-western-blot.jpg)
Western blot - Anti-GNS antibody [EPR8329(2)] (ab154177)All lanes : Anti-GNS antibody [EPR8329(2)] (ab154177) at 1/10000 dilution
Lane 1 : PC3 cell lysate
Lane 2 : NCI-H460 cell lysate
Lane 3 : 293T cell lysate
Lysates/proteins at 10 µg per lane.
Secondary
All lanes : Goat anti-rabbit HRP at 1/2000 dilution
Predicted band size: 62 kDa
Datasheets and documents
References
ab154177 has not yet been referenced specifically in any publications.
