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Mouse Anti-SUMF1 Recombinant Antibody (CBXS-1776) (CBMAB-S4593-CQ)

This product is a mouse antibody that recognizes SUMF1. The antibody CBXS-1776 can be used for immunoassay techniques such as: WB, IP.
See all SUMF1 antibodies

Summary

Host Animal
Mouse
Specificity
Human, Mouse
Clone
CBXS-1776
Antibody Isotype
IgG2b
Application
WB, IP

Basic Information

Immunogen
NS0-derived recombinant human Sulfatase Modifying Factor 1/SUMF1 isoform 1, Ser34-Asp374 (Ser63Asn), Accession # Q8NBK3.3
Specificity
Human, Mouse
Antibody Isotype
IgG2b
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Lyophilized
Concentration
LYOPH
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Sulfatase Modifying Factor 1
Introduction
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.
Entrez Gene ID
Human285362
Mouse58911
UniProt ID
HumanQ8NBK3
MouseQ8R0F3
Alternative Names
AAPA3037; FGE; UNQ3037
Function
Oxidase that catalyzes the conversion of cysteine to 3-oxoalanine on target proteins, using molecular oxygen and an unidentified reducing agent (PubMed:12757706, PubMed:15657036, PubMed:15907468, PubMed:25931126, PubMed:16368756, PubMed:21224894).
3-oxoalanine modification, which is also named formylglycine (fGly), occurs in the maturation of arylsulfatases and some alkaline phosphatases that use the hydrated form of 3-oxoalanine as a catalytic nucleophile (PubMed:12757706, PubMed:15657036, PubMed:15907468, PubMed:25931126, PubMed:16368756).
Known substrates include GALNS, ARSA, STS and ARSE (PubMed:12757706, PubMed:15907468, PubMed:15657036).
Biological Process
Biological Process glycosphingolipid metabolic processTAS:Reactome
Biological Process post-translational protein modificationManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process protein oxidationManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Endoplasmic reticulum lumen
Involvement in disease
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.
PTM
N-glycosylated. Contains high-mannose-type oligosaccharides.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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