Immunohistochemistry (IHC)
(Human Lung )
Rabbit GUSB Polyclonal Antibody | anti-GUSB antibody
GUSB antibody - C-terminal region
Immunohistochemistry (IHC)
(Human Lung )
Immunohistochemistry (IHC)
(Human Lung )
Immunohistochemistry (IHC)
(Rabbit Anti-GUSB AntibodyParaffin Embedded Tissue: Human alveolar cellCellular Data: Epithelial cells of renal tubuleAntibody Concentration: 4.0-8.0 ug/mlMagnification: 400X)
Immunohistochemistry (IHC)
(Rabbit Anti-GUSB AntibodyParaffin Embedded Tissue: Human alveolar cellCellular Data: Epithelial cells of renal tubuleAntibody Concentration: 4.0-8.0 ug/mlMagnification: 400X)
Western Blot (WB)
(WB Suggested Anti-GUSB Antibody Titration: 2.5ug/mlPositive Control: HepG2 cell lysate)
Western Blot (WB)
(WB Suggested Anti-GUSB Antibody Titration: 2.5ug/mlPositive Control: HepG2 cell lysate)
Target Description: GUSB plays an important role in the degradation of dermatan and keratan sulfates.
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a hydrolase that degrades glycosaminoglycans, including heparan sulfate, dermatan sulfate, and chondroitin-4,6-sulfate. The enzyme forms a homotetramer that is localized to the lysosome. Mutations in this gene result in mucopolysaccharidosis type VII. Alternative splicing results in multiple transcript variants. There are many pseudogenes of this locus in the human genome.[provided by RefSeq, May 2014]
Uniprot Description
GUSB: Plays an important role in the degradation of dermatan and keratan sulfates. Defects in GUSB are the cause of mucopolysaccharidosis type 7 (MPS7); also known as Sly syndrome. MPS7 is an autosomal recessive lysosomal storage disease characterized by inability to degrade glucuronic acid-containing glycosaminoglycans. The phenotype is highly variable, ranging from severe lethal hydrops fetalis to mild forms with survival into adulthood. Most patients with the intermediate phenotype show hepatomegaly, skeletal anomalies, coarse facies, and variable degrees of mental impairment. Mucopolysaccharidosis type 7 is associated with non- immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders. Belongs to the glycosyl hydrolase 2 family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Carbohydrate Metabolism - starch and sucrose; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Hydrolase; EC 3.2.1.31; Xenobiotic Metabolism - drug metabolism - other enzymes; Carbohydrate Metabolism - pentose and glucuronate interconversions; Glycan Metabolism - glycosaminoglycan degradation
Chromosomal Location of Human Ortholog: 7q21.11
Cellular Component: lysosomal lumen; membrane; intracellular membrane-bound organelle
Molecular Function: protein domain specific binding; beta-glucuronidase activity; receptor binding
Biological Process: glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis; hyaluronan metabolic process; hyaluronan catabolic process
Disease: Mucopolysaccharidosis, Type Vii
Research Articles on GUSB
Similar Products
Product Notes
The GUSB gusb (Catalog #AAA3207271) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The GUSB antibody - C-terminal region reacts with Cow, Dog, Guinea Pig, Horse, Human, Mouse, Rabbit, Rat, Zebrafish and may cross-react with other species as described in the data sheet. AAA Biotech's GUSB can be used in a range of immunoassay formats including, but not limited to, Immunohistochemistry (IHC), Western Blot (WB). Researchers should empirically determine the suitability of the GUSB gusb 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: VLGNKKGIFT RQRQPKSAAF LLRERYWKIA NETRYPHSVA KSQCLENSPF. It is sometimes possible for the material contained within the vial of "GUSB, 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
If you are ready to order, navigate to Shopping Cart and get ready to checkout.
