product summary
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company name :
MyBioSource
product type :
antibody
product name :
SUMF1 Rabbit Polyclonal
catalog :
MBS7604620
quantity :
0.1 mg
price :
290 USD
clonality :
polyclonal
host :
rabbit
conjugate :
nonconjugated
reactivity :
human, mouse
application :
western blot, ELISA, enzyme immunoassay
more info or order :
image
image 1 :
mouse liver tissue were subjected to SDS PAGE followed by western blot with MBS7604620 (SUMF1 antibody) at dilution of 1:1000
product information
catalog number :
MBS7604620
products type :
Antibody
products full name :
SUMF1 Rabbit Polyclonal
products short name :
[SUMF1]
products name syn :
[AAPA3037, FGE, sulfatase modifying factor 1, SUMF1, UNQ3037/PRO9852]
other names :
[sulfatase-modifying factor 1 isoform 3; Sulfatase-modifying factor 1; sulfatase-modifying factor 1; sulfatase modifying factor 1; C-alpha-formylglycine-generating enzyme 1]
products gene name :
[SUMF1]
other gene names :
[SUMF1; SUMF1; FGE; UNQ3037; AAPA3037; FGE]
uniprot entry name :
SUMF1_HUMAN
clonality :
Polyclonal
isotype :
IgG
host :
Rabbit
reactivity :
Human, Mouse
sequence length :
354
specificity :
Human, Mouse; other species are not tested. Please decide the specificity by homology.
purity :
>=95% as determined by SDS-PAGE. Immunogen Affinity Purified
form :
Liquid
storage stability :
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)
tested application :
ELISA (EIA), Western Blot (WB)
app notes :
WB: 1:500-1:2000
image1 heading :
Western Blot
other info1 :
Immunogen: Sulfatase modifying factor 1
products description :
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.
ncbi gi num :
257470977
ncbi acc num :
NP_001158147.1
ncbi gb acc num :
NM_001164675.1
ncbi mol weight :
46 kDa
ncbi pathways :
Gamma Carboxylation, Hypusine Formation And Arylsulfatase Activation Pathway (1268702); Glycosphingolipid Metabolism Pathway (1270099); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Pathway (1269956); Metabolism Of Lipids And Lipoproteins Pathway (1270001); Metabolism Of Proteins Pathway (1268677); Post-translational Protein Modification Pathway (1268701); Sphingolipid Metabolism Pathway (1270097); The Activation Of Arylsulfatases Pathway (1268709)
ncbi summary :
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 summary :
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: Oxidoreductase; Secreted; EC 1.8.99.-; Endoplasmic reticulum; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 3p26.1. Cellular Component: endoplasmic reticulum lumen. Molecular Function: metal ion binding; oxidoreductase activity; protein homodimerization activity. Biological Process: glycosphingolipid metabolic process; post-translational protein modification. Disease: Multiple Sulfatase Deficiency
size1 :
0.1 mg
price1 :
290 USD
more info or order :
company information
MyBioSource
P.O. Box 153308
San Diego, CA 92195-3308
San Diego, CA 92195-3308
sales@mybiosource.com
https://www.mybiosource.com1-888-627-0165
headquarters: USA
MyBioSource, LLC was orginally founded in Vancouver by three enthusiastic scientists who are passionate about providing the world with the best reagents available. Together, they form a company with a big vision known as MyBioSource. MyBioSource is now located in San Diego, California, USA.
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