Recombinant Anti-GNS antibody [EPR8329(2)] (ab154177)
![Western blot - Anti-GNS antibody [EPR8329(2)] (ab154177)](https://www.abcam.com/ps/products/154/ab154177/Images/ab154177-389432-anti-gns-antibody-epr83292-western-blot-human-knockout-lysate.jpg "Western blot - Anti-GNS antibody [EPR8329(2)] (ab154177)")
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR8329(2)] to GNS
- Suitable for: WB
- Knockout validated
- Reacts with: Human
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 & Species
See all applications and species dataApplication Species WB Human
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Immunogen
Synthetic peptide, corresponding to residues in Human GNS (UniProt: P15586).
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Positive control
- WB: HeLa, 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.
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at -20ºC. -
Storage buffer
pH: 7.2
Preservative: 0.01% Sodium azide
Constituents: 9% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA, 50% Tissue culture supernatant -
Concentration information loading... -
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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KO cell lines
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KO cell lysates
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Recombinant Protein
Applications
The Abpromise guarantee
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.
Tested applications are guaranteed to work and covered by our Abpromise guarantee.
Predicted to work for this combination of applications and species but not guaranteed.
Does not work for this combination of applications and species.
| Application | Species |
|---|---|
| WB |
Human
|
| Application | Abreviews | Notes |
|---|---|---|
| WB |
1/10000 - 1/50000. Predicted molecular weight: 62 kDa.
|
| Notes |
|---|
|
WB
1/10000 - 1/50000. Predicted molecular weight: 62 kDa. |
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
-
Western blot - Anti-GNS antibody [EPR8329(2)] (ab154177)All lanes : Anti-GNS antibody [EPR8329(2)] (ab154177) at 1/1000 dilution
Lane 1 : Wild-type HeLa cell lysate
Lane 2 : GNS knockout HeLa cell lysate
Lane 3 : 293T cell lysate
Lysates/proteins at 20 µg per lane.
Secondary
All lanes : Goat anti-Rabbit IgG H&L (IRDye® 800CW) preadsorbed (ab216773) at 1/10000 dilution
Predicted band size: 62 kDa
Observed band size: 90 kDa why is the actual band size different from the predicted?Lanes 1-3: Merged signal (red and green). Green - ab154177 observed at 90 kDa. Red - loading control ab7291 observed at 50 kDa.
ab154177 Anti-GNS antibody [EPR8329(2)] was shown to specifically react with GNS in wild-type HeLa cells. Loss of signal was observed when knockout cell line ab265495 (knockout cell lysate ab257975) was used. Wild-type and GNS knockout samples were subjected to SDS-PAGE. ab154177 and Anti-alpha Tubulin antibody [DM1A] - Loading Control (ab7291) were incubated overnight at 4°C at 1 in 1000 dilution and 1 in 20000 dilution respectively. Blots were developed with Goat anti-Rabbit IgG H&L (IRDye® 800CW) preadsorbed (ab216773) and Goat anti-Mouse IgG H&L (IRDye® 680RD) preadsorbed (ab216776) secondary antibodies at 1 in 20000 dilution for 1 hour at room temperature before imaging.
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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 -
![Anti-GNS antibody [EPR8329(2)] (ab154177)](https://www.abcam.com/ps/products/154/ab154177/Images/ab154177-3-benefits-of-recombinant-antibodies.png)
Anti-GNS antibody [EPR8329(2)] (ab154177)
Datasheets and documents
References (0)
ab154177 has not yet been referenced specifically in any publications.
