Typical Testing Data/Standard Curve (for reference only)
(Fig.1. Human Sulfatase-modifying factor 1 (SUMF1) Standard Curve.)
Human Sulfatase-Modifying Factor 1 (SUMF1) ELISA Kit | SUMF1 elisa kit
Human Sulfatase-Modifying Factor 1 (SUMF1) ELISA Kit
* Allow all reagents to warm to room temperature for at least 30 minutes before opening.
* Pre-rinse the pipet tip with reagent, use fresh pipet tips for each sample, standard and reagent to avoid contamination.
* Unused wells must be kept desiccated at 4 degree C in the sealed bag provided.
* Mix Thoroughly is very important for the result. It is recommended using low frequency oscillator or slight hand shaking every 10 minutes.
* It is recommended that all samples and standards be assayed in duplicate or triplicate.
Typical Testing Data/Standard Curve (for reference only)
(Fig.1. Human Sulfatase-modifying factor 1 (SUMF1) Standard Curve.)
Typical Testing Data/Standard Curve (for reference only)
(Fig.1. Human Sulfatase-modifying factor 1 (SUMF1) Standard Curve.)
Background: Sulfatases catalyze the hydrolysis of sulfate esters such as glycosaminoglycans, sulfolipids, and steroid sulfates. C-alpha-formylglycine (FGly), the catalytic residue in the active site of eukaryotic sulfatases, is posttranslationally generated from a cysteine by SUMF1, the FGly-generating enzyme (FGE), in the endoplasmic reticulum (ER). The genetic defect of FGly formation caused by mutations in the SUMF1 gene results in multiple sulfatase deficiency (MSD), a lysosomal storage disorder (Roeser et al., 2006).Highly expressed in kidney, pancreas and liver. Detected at lower levels in leukocytes, lung, placenta, small intestine, skeletal muscle and heart.
NCBI and Uniprot Product Information
NCBI Description
This gene encodes an enzyme that catalyzes the hydrolysis of sulfate esters by oxidizing a cysteine residue in the substrate sulfatase to an active site 3-oxoalanine residue, which is also known as C-alpha-formylglycine. Mutations in this gene cause multiple sulfatase deficiency, a lysosomal storage disorder. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2009]
Uniprot Description
SUMF1: Using molecular oxygen and an unidentified reducing agent, oxidizes a cysteine residue in the substrate sulfatase to an active site 3-oxoalanine residue, which is also called C(alpha)-formylglycine. Known substrates include GALNS, ARSA, STS and ARSE. Defects in SUMF1 are the cause of multiple sulfatase deficiency (MSD). MSD is 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. Inheritance is autosomal recessive. Belongs to the sulfatase-modifying factor family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 1.8.99.-; Secreted, signal peptide; Oxidoreductase; Endoplasmic reticulum; Secreted
Chromosomal Location of Human Ortholog: 3p26.1
Cellular Component: endoplasmic reticulum lumen
Molecular Function: protein homodimerization activity; metal ion binding; oxidoreductase activity
Biological Process: sphingolipid metabolic process; cellular protein metabolic process; glycosphingolipid metabolic process; post-translational protein modification
Disease: Multiple Sulfatase Deficiency
Research Articles on SUMF1
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Product Notes
The Human SUMF1 sumf1 (Catalog #AAA9714069) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9714069 ELISA Kit recognizes Human SUMF1. It is sometimes possible for the material contained within the vial of "Sulfatase-Modifying Factor 1 (SUMF1), ELISA Kit" 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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