Human Sulfamidase/SGSH Alexa Fluor™ Plus 594-conjugated Antibody

R&D Systems, part of Bio-Techne | Catalog # FAB8380AFP594

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FAB8380AFP594-100UG
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Key Product Details

Species Reactivity

Human

Applications

Immunocytochemistry

Label

Alexa Fluor Plus 594 (Excitation = 590 nm, Emission = 618 nm)

Antibody Source

Monoclonal Mouse IgG2A Clone # 1018387

View all Sulfamidase/SGSH Antibodies »

Product Specifications

Immunogen

Spodoptera frugiperda, Sf 21 (baculovirus)-derived human Sulfamidase/SGSH protein

Specificity

Detects human Sulfamidase/SGSH in direct ELISAs.

Clonality

Monoclonal

Host

Mouse

Isotype

IgG2A

Applications

Application
Recommended Usage

Immunocytochemistry

Optimal dilution of this antibody should be experimentally determined.

Formulation, Preparation, and Storage

Formulation

Supplied 0.2 mg/mL in a saline solution containing BSA and Sodium Azide.

Shipping

The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.

Stability & Storage

Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied

Background: Sulfamidase/SGSH

Also known as N-sulfoglucosamine sulfohydrolase and heparan N-sulfatase, Sulfamidase/SGSH is an important member of the sulfatase family involved in the degradation of heparan sulfate (HS) (1). Different from the HS specific endosulfatases that remove sulfate from internal GlcNAc residues (2), SGSH removes sulfate group from the non-reducing end glucosamine residues on HS. The SGSH deficiency results in mucopolysaccharidosis type IIIA (MPS IIIA, Sanfilippo A syndrome), an autosomal recessive lysosomal storage disease characterized by neurological dysfunction but relatively mild somatic manifestations (3). Human SGSH shows 88.6% sequence identity with that of mouse sequence.

References

  1. DiezRoux, G. and Ballabio, A. et al. (2005) Annu. Rev. Genomics Hum. Genet. 6:355.
  2. Morimoto-Tomita, M. et al. (2002) J. Biol. Chem. 277:49175.
  3. Blanch, L. et al. (1997) Hum. Mol. Genet. 6:787.

Alternate Names

HSS, MPS3A, SFMD, SGSH

Entrez Gene IDs

6448 (Human); 27029 (Mouse)

Gene Symbol

SGSH

UniProt

P51688

Additional Sulfamidase/SGSH Products

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Product Datasheets

Certificate of Analysis

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Note: Certificate of Analysis not available for kit components.

Product Specific Notices


This product is provided under an intellectual property license from Life Technologies Corporation. The transfer of this product is conditioned on the buyer using the purchased product solely in research conducted by the buyer, excluding contract research or any fee for service research, and the buyer must not (1) use this product or its components for (a) diagnostic, therapeutic or prophylactic purposes; (b) testing, analysis or screening services, or information in return for compensation on a per-test basis; or (c) manufacturing or quality assurance or quality control, and/or (2) sell or transfer this product or its components for resale, whether or not resold for use in research. For information on purchasing a license to this product for purposes other than as described above, contact Life Technologies Corporation, 5781 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@thermofisher.com.

For research use only

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