Rabbit GNS Polyclonal Antibody | anti-GNS antibody
GNS antibody - C-terminal region
WB (Western Blot)
(WB Suggested Anti-GNS Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:312500Positive Control: Human Liver)
IHC (Immunohiostchemistry)
(Rabbit Anti-GNS AntibodyFormalin Fixed Paraffin Embedded Tissue: Human Liver TissueObserved Staining: Cytoplasm in Kupffer cells in sinusoidsPrimary Antibody Concentration: 1:100Other Working Concentrations: N/ASecondary Antibody: Donkey anti-Rabbit-Cy3Secondary Antibody Concentration: 1:200Magnification: 20XExposure Time: 0.5 - 2.0 sec)
IHC (Immunohistochemistry)
(Rabbit Anti-GNS AntibodyFormalin Fixed Paraffin Embedded Tissue: Human heart TissueObserved Staining: CytoplasmicPrimary Antibody Concentration: 1:100Other Working Concentrations: N/ASecondary Antibody: Donkey anti-Rabbit-Cy3Secondary Antibody Concentration: 1:200Magnification: 20XExposure Time: 0.5 - 2.0 sec)
Target Description: GNS is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparin sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder ucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome.The product of this gene is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome.
NCBI and Uniprot Product Information
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Product Notes
The GNS gns (Catalog #AAA199532) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The GNS antibody - C-terminal region reacts with Cow, Dog, Goat, Guinea Pig, Horse, Human, Mouse, Rabbit, Rat, Zebrafish and may cross-react with other species as described in the data sheet. AAA Biotech's GNS can be used in a range of immunoassay formats including, but not limited to, IHC (Immunohistochemistry), WB (Western Blot). Researchers should empirically determine the suitability of the GNS gns for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. The amino acid sequence is listed below: Synthetic peptide located within the following region: PILRGASNLT WRSDVLVEYQ GEGRNVTDPT CPSLSPGVSQ CFPDCVCEDA. It is sometimes possible for the material contained within the vial of "GNS, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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