SUMF1, Polyclonal Antibody

Sulfatase-modifying factor 1; 1899-; C-alpha-formylglycine-generating enzyme 1; SUMF1; FGE

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This SUMF1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 303-331 amino acids from the C-terminal region of human SUMF1.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-SUMF1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

SUMF1 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.

Signal Transduction

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC), Flow Cytometry (FC/FACS)

WB~~1:1000

Western blot analysis of SUMF1 Antibody (C-Term) in Hela cell line lysates (35ug/lane). SUMF1 (arrow) was detected using the purified Pab.

SUMF1 Antibody (C-Term) immunohistochemistry analysis in formalin fixed and paraffin embedded human pancreas tissue followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the SUMF1 Antibody (C-Term) for immunohistochemistry. Clinical relevance has not been evaluated.

SUMF1 Antibody (C-Term) flow cytometric analysis of Hela cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.

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sulfatase modifying factor 1

sulfatase-modifying factor 1

Sulfatase-modifying factor 1

FGE  [Similar Products]

SUMF1_HUMAN

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]

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: Secreted; Endoplasmic reticulum; EC 1.8.99.-; Oxidoreductase; Secreted, signal peptide

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

Oshikawa, M., et al. Mol. Vis. 15, 482-494 (2009)
Fraldi, A., et al. Hum. Mol. Genet. 17(17):2610-2621(2008)
Hara, K., et al. Neurology 71(8):547-551(2008)
Yis, U., et al. Brain Dev. 30(5):374-377(2008)

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