Anti-GALNS Antibody Picoband® Fluoro647 Conjugated
Product Specifications
Background
N-acetylgalactosamine-6-sulfatase is an enzyme that, in humans, is encoded by the GALNS gene. This gene encodes N-acetylgalactosamine-6-sulfatase which is a lysosomal exohydrolase required for the degradation of the glycosaminoglycans, keratan sulfate, and chondroitin 6-sulfate. Sequence alterations including point, missense and nonsense mutations, as well as those that affect splicing, result in a deficiency of this enzyme. Deficiencies of this enzyme lead to Morquio A syndrome, a lysosomal storage disorder.
Synonyms
N-acetylgalactosamine-6-sulfatase;3.1.6.4; Chondroitinsulfatase; Chondroitinase; Galactose-6-sulfate sulfatase; GalN6S; N-acetylgalactosamine-6-sulfate sulfatase; GalNAc6S sulfatase; GALNS
Gene Name
GALNS
Gene ID
2588
UniProt
P34059
Host
Rabbit
Reactivity
Human, Mouse, Rat
Cross Reactivity
No cross-reactivity with other proteins.
Immunogen
E. coli-derived human GALNS recombinant protein (Position: Y181-N289) .
Clonality
Polyclonal
Tissue Specificity
Highly expressed in spleen, thymus, small intestine and peripheral blood leukocytes. Expressed in resting B- cells and activated T-cells, but not in resting T-cells.
Applications
Flow Cytometry
Field of Research
Cell Biology, Cytoskeleton/ECM, ECM Proteins, Extracellular Matrix, Organelles, Proteolysis/Ubiquitin, Proteolytic Enzymes, Signal Transduction, Subcellular Markers, Tags & Cell Markers
Purification
Immunogen affinity purified.
Form
Liquid
Function
Receptor for TNFSF13/APRIL and TNFSF13B/TALL1/BAFF/BLYS that binds both ligands with similar high affinity. Mediates calcineurin-dependent activation of NF-AT, as well as activation of NF-kappa-B and AP-1. Involved in the stimulation of B- and T- cell function and the regulation of humoral immunity.
References & Citations
1. Entrez Gene: GALNS galactosamine (N-acetyl) -6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA) . 2. Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al. (Jan 1992) . Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase. Biochem Biophys Res Commun. 181 (2) : 677–83.
Storage Conditions
At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.
Calculated Molecular Weight
58026 MW
Specificity
No cross reactivity with other proteins.
Applications Notes
6
Gene Name Synonym
N-acetylgalactosamine-6-sulfatase
Subcellular Location
Lysosome.
Protein Name
Peroxisomal multifunctional enzyme type 2
Isotype
Rabbit IgG
Contents
Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.
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