Anti-GNS antibody (ab230044)
Key features and details
- Rabbit polyclonal to GNS
- Suitable for: WB, IHC-P
- Reacts with: Mouse, Human
- Isotype: IgG
Overview
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Product name
Anti-GNS antibody
See all GNS primary antibodies -
Description
Rabbit polyclonal to GNS -
Host species
Rabbit -
Tested applications
Suitable for: WB, IHC-Pmore details -
Species reactivity
Reacts with: Mouse, Human
Predicted to work with: Goat, Cow -
Immunogen
Recombinant fragment corresponding to Human GNS aa 250 to the C-terminus.
Database link: P15586 -
Positive control
- WB: Mouse liver tissue lysate. IHC-P: Human testis and kidney tissues.
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General notes
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. -
Storage buffer
pH: 7.30
Preservative: 0.02% Sodium azide
Constituents: PBS, 50% Glycerol (glycerin, glycerine) -
Concentration information loading...
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Purity
Immunogen affinity purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
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Positive Controls
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Recombinant Protein
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Related Products
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab230044 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 |
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WB |
1/1000 - 1/5000. Predicted molecular weight: 63,60 kDa.
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IHC-P |
1/20 - 1/200.
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Notes |
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WB
1/1000 - 1/5000. Predicted molecular weight: 63,60 kDa. |
IHC-P
1/20 - 1/200. |
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
- Entrez Gene: 75612 Mouse
- Omim: 607664 Human
- SwissProt: P15586 Human
- SwissProt: Q8BFR4 Mouse
- Unigene: 334534 Human
- Unigene: 207683 Mouse
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Alternative names
- 2610016K11Rik antibody
- AU042285 antibody
- C87209 antibody
see all
Images
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Anti-GNS antibody (ab230044) at 1/1000 dilution + Mouse liver tissue lysate
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 63,60 kDa
Observed band size: 62,27,37 kDa why is the actual band size different from the predicted? -
Paraffin-embedded human kidney tissue stained for GNS using ab230044 at 1/100 dilution in immunohistochemical analysis.
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Paraffin-embedded human testis tissue stained for GNS using ab230044 at 1/100 dilution in immunohistochemical analysis.
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
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SDS download
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Datasheet download
References (0)
ab230044 has not yet been referenced specifically in any publications.