Western Blot (WB)
(Western Blot: Sample: Porcine Kidney lysate; Primary Ab: 1ug/ml Rabbit Anti-Human ARSB Antibody;Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody;)
Rabbit anti-Human Arylsulfatase B Polyclonal Antibody | anti-ARSB antibody
Arylsulfatase B (ARSB) Polyclonal Antibody
Immunohistochemistry in formalin fixed frozen section: 1:50-500
Enzyme-linked Immunosorbent Assay: 1:100-200
Usage: 10uL per well when 3,3'-Diaminobenzidine(DAB) as the substrate.
5uL per well when used in enhanced chemilumescent (ECL).
Note: The quality control is specifically manufactured as the positive control.Not used for other purposes.
Loading Buffer: 100mM Tris(pH8.8), 2% SDS, 200mM NaCl, 50% glycerol,BPB 0.01%, NaN3 0.02%.
Western Blot (WB)
(Western Blot: Sample: Porcine Kidney lysate; Primary Ab: 1ug/ml Rabbit Anti-Human ARSB Antibody;Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody;)
Western Blot (WB)
(Western Blot: Sample: Porcine Kidney lysate; Primary Ab: 1ug/ml Rabbit Anti-Human ARSB Antibody;Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody;)
Western Blot (WB)
(Western Blot: Sample: Recombinant protein.)
Western Blot (WB)
(Western Blot: Sample: Recombinant protein.)
Immunohistochemistry (IHC)
(DAB staining on IHC-P. Samples: Human Tissue))
Immunohistochemistry (IHC)
(DAB staining on IHC-P. Samples: Human Tissue))
NCBI and Uniprot Product Information
NCBI Description
Arylsulfatase B encoded by this gene belongs to the sulfatase family. The arylsulfatase B homodimer hydrolyzes sulfate groups of N-Acetyl-D-galactosamine, chondriotin sulfate, and dermatan sulfate. The protein is targeted to the lysozyme. Mucopolysaccharidosis type VI is an autosomal recessive lysosomal storage disorder resulting from a deficiency of arylsulfatase B. Two alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. [provided by RefSeq, Dec 2016]
Uniprot Description
ARSB: Defects in ARSB are the cause of mucopolysaccharidosis type 6 (MPS6); also known as Maroteaux-Lamy syndrome. MPS6 is an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of dermatan sulfate. Clinical features can include abnormal growth, short stature, stiff joints, skeletal malformations, corneal clouding, hepatosplenomegaly, and cardiac abnormalities. A wide variation in clinical severity is observed. Arylsulfatase B activity is defective in multiple sulfatase deficiency (MSD). A clinically and biochemically heterogeneous disorder caused by the simultaneous impairment of all sulfatases, due to defective post-translational modification and activation. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay. Arylsulfatase B activity is impaired in multiple sulfatase deficiency due to mutations in SUMF1. SUMF1 mutations result in defective post-translational modification of ARSB at residue Cys- 91 that is not converted to 3-oxoalanine. Belongs to the sulfatase family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 3.1.6.12; Glycan Metabolism - glycosaminoglycan degradation; Hydrolase
Chromosomal Location of Human Ortholog: 5q14.1
Cellular Component: cell surface; endoplasmic reticulum lumen; extracellular region; Golgi apparatus; lysosomal lumen; lysosome; mitochondrion; rough endoplasmic reticulum
Molecular Function: arylsulfatase activity; metal ion binding; N-acetylgalactosamine-4-sulfatase activity
Biological Process: autophagy; central nervous system development; chondroitin sulfate catabolic process; glycosphingolipid metabolic process; lysosomal transport; lysosome organization and biogenesis; neutrophil degranulation; post-translational protein modification; response to estrogen; response to methylmercury; response to nutrient; response to pH
Disease: Mucopolysaccharidosis Type Vi
Research Articles on ARSB
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Product Notes
The ARSB arsb (Catalog #AAA2032627) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Arylsulfatase B (ARSB) Polyclonal Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Arylsulfatase B can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA). Western Blot: 1:50-400 Immunohistochemistry in formalin fixed frozen section: 1:50-500 Enzyme-linked Immunosorbent Assay: 1:100-200. Researchers should empirically determine the suitability of the ARSB arsb 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. It is sometimes possible for the material contained within the vial of "Arylsulfatase B, 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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