Recombinant Anti-SGSH/HSS antibody [EPR17312] (ab200346)
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR17312] to SGSH/HSS
- Suitable for: ICC/IF, WB
- Reacts with: Human
Overview
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Product name
Anti-SGSH/HSS antibody [EPR17312]
See all SGSH/HSS primary antibodies -
Description
Rabbit monoclonal [EPR17312] to SGSH/HSS -
Host species
Rabbit -
Tested Applications & Species
Application Species ICC/IF HumanWB Human -
Immunogen
Recombinant fragment. This information is proprietary to Abcam and/or its suppliers.
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Positive control
- WB: HepG2 and MCF7 whole cell lysates; Human prostate cancer lysate; ICC/IF: MCF7 and HepG2 cells.
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General notes
This product was previously labelled as SGSH
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 +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. -
Storage buffer
pH: 7.2
Preservative: 0.01% Sodium azide
Constituents: 59% PBS, 40% Glycerol, 0.05% BSA -
Concentration information loading...
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Purity
Protein A purified -
Clonality
Monoclonal -
Clone number
EPR17312 -
Isotype
IgG -
Research areas
Associated products
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Alternative Versions
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Compatible Secondaries
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Isotype control
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Recombinant Protein
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab200346 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 |
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ICC/IF |
Human
|
WB |
Human
|
Application | Abreviews | Notes |
---|---|---|
ICC/IF |
1/250.
|
|
WB |
1/1000. Detects a band of approximately 57 kDa (predicted molecular weight: 57 kDa).
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Notes |
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ICC/IF
1/250. |
WB
1/1000. Detects a band of approximately 57 kDa (predicted molecular weight: 57 kDa). |
Target
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Involvement in disease
Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A) [MIM:252900]; also known as Sanfilippo syndrome A. MPS3A is a severe 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. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival. -
Sequence similarities
Belongs to the sulfatase family. -
Post-translational
modificationsThe 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: 6448 Human
- Omim: 605270 Human
- SwissProt: P51688 Human
- Unigene: 31074 Human
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Alternative names
- Heparan sulfate sulfatase antibody
- Heparan sulphate sulphatase antibody
- HSS antibody
see all
Images
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Anti-SGSH/HSS antibody [EPR17312] (ab200346) at 1/1000 dilution + HepG2 (Human liver hepatocellular carcinoma) whole cell lysate at 20 µg
Secondary
Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/1000 dilution
Predicted band size: 57 kDa
Observed band size: 57 kDa
Exposure time: 3 minutesBlocking/Dilution buffer: 5% NFDM/TBST.
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Anti-SGSH/HSS antibody [EPR17312] (ab200346) at 1/10000 dilution + MCF7 (Human breast adenocarcinoma cell line) whole cell lysate at 10 µg
Secondary
Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/1000 dilution
Predicted band size: 57 kDa
Observed band size: 57 kDa
Exposure time: 3 minutesBlocking/Dilution buffer: 5% NFDM/TBST.
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Anti-SGSH/HSS antibody [EPR17312] (ab200346) at 1/10000 dilution + Human prostate cancer lysate at 10 µg
Secondary
Anti-Rabbit IgG (HRP), specific to the non-reduced form of IgG at 1/1000 dilution
Predicted band size: 57 kDa
Observed band size: 57 kDa
Exposure time: 30 secondsBlocking/Dilution buffer: 5% NFDM/TBST.
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Immunofluorescent analysis of 100% methanol-fixed, 0.1% Triton X-100 permeabilized MCF7 (Human breast adenocarcinoma cell line) cells labeling SGSH/HSS with ab200346 at 1/250 dilution, followed by Goat anti-rabbit IgG (Alexa Fluor® 488) (ab150077) secondary antibody at 1/500 dilution (green). Cytoplasm staining on MCF7 cell line was observed. The nuclear counter stain is DAPI (blue). Tubulin is detected with ab7291 (anti-Tubulin mouse mAb) at 1/1000 dilution and ab150120 (AlexaFluor®594 Goat anti-Mouse secondary) at 1/500 dilution (red).
The negative controls are as follows:
-ve control 1: ab200346 at 1/250 dilution followed by ab150120 (AlexaFluor®594 Goat anti-Mouse secondary) at 1/500 dilution.
-ve control 2: ab7291 (anti-Tubulin mouse mAb) at 1/1000 dilution followed by ab150077 (Alexa Fluor®488 Goat Anti-Rabbit IgG H&L) at 1/500 dilution. -
Immunofluorescent analysis of 100% methanol-fixed, 0.1% Triton X-100 permeabilized HepG2 (Human liver hepatocellular carcinoma) cells labeling SGSH/HSS with ab200346 at 1/250 dilution, followed by Goat anti-rabbit IgG (Alexa Fluor® 488) (ab150077) secondary antibody at 1/500 dilution (green). Cytoplasm staining on HepG2 cell line was observed. The nuclear counter stain is DAPI (blue). Tubulin is detected with ab7291 (anti-Tubulin mouse mAb) at 1/1000 dilution and ab150120 (AlexaFluor®594 Goat anti-Mouse secondary) at 1/500 dilution (red).
The negative controls are as follows:
-ve control 1: ab200346 at 1/250 dilution followed by ab150120 (AlexaFluor®594 Goat anti-Mouse secondary) at 1/500 dilution.
-ve control 2: ab7291 (anti-Tubulin mouse mAb) at 1/1000 dilution followed by ab150077 (Alexa Fluor®488 Goat Anti-Rabbit IgG H&L) at 1/500 dilution.
Protocols
Datasheets and documents
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SDS download
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Datasheet download
Certificate of Compliance
References (1)
ab200346 has been referenced in 1 publication.
- Knottnerus SJG et al. Prediction of phenotypic severity in mucopolysaccharidosis type IIIA. Ann Neurol 82:686-696 (2017). WB, ICC/IF ; Human . PubMed: 29023963