Rabbit anti-Human GALNS Polyclonal Antibody | anti-GALNS antibody

GALNS, ID (GALNS, N-acetylgalactosamine-6-sulfatase, Chondroitinsulfatase, Galactose-6-sulfate sulfatase, N-acetylgalactosamine-6-sulfate sulfatase) (APC)

Cat. Num. AAA6301083

• Gene Names: GALNS; GAS; MPS4A; GalN6S; GALNAC6S
• Reactivity: Human
• Applications: Flow Cytometry, Functional Assay, Immunofluorescence, Immunohistochemistry, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: GALNS; Polyclonal Antibody; ID (GALNS; N-acetylgalactosamine-6-sulfatase; Chondroitinsulfatase; Galactose-6-sulfate sulfatase; N-acetylgalactosamine-6-sulfate sulfatase) (APC); N-acetylgalactosamine-6-sulfate sulfatase; anti-GALNS antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Allophycocyanin (APC).

• Applicable Applications for anti-GALNS antibody: Flow Cytometry (FC/FACS), FLISA, Immunofluorescence (IF), Immunohistochemistry (IHC), Western Blot (WB)
• Application Notes: FC/FACS: 1:10-50; FLISA: 1:1,000; IF: 1:10-50; IHC: 1:10-50; WB: 1:100-500; Applications are based on unconjugated antibody.
• Immunogen: GALNS antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 243-271 amino acids from the Central region of human GALNS.
• Conjugate: APC
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: APC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(GALNS Antibody (Center) western blot analysis in MCF-7 cell line lysates (35ug/lane).This demonstrates the GALNS antibody detected the GALNS protein (arrow).)

Immunohistochemistry (IHC)

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

Immunofluorescence (IF)

(Confocal immunofluorescent analysis of GALNS Antibody (Center) with MCF-7 cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green).DAPI was used to stain the cell nuclear (blue).)

Flow Cytometry (FC/FACS)

(GALNS Antibody (Center) flow cytometric analysis of MCF-7 cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Related Product Information for anti-GALNS antibody

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. [provided by RefSeq].

Product Categories/Family for anti-GALNS antibody

Antibodies; Enzymes

References

Rose, J.E., et al. Mol. Med. 16 (7-8), 247-253 (2010): Bank, R.A., et al. Mol. Genet. Metab. 97(3):196-201(2009). Yan, S., et al. Biopolymers 92(5):399-404(2009). Carraresi, L., et al. Clin. Chim. Acta 397 (1-2), 72-76 (2008): Bhattacharyya, S., et al. J. Biol. Chem. 283(15):9523-9530(2008).

NCBI and Uniprot Product Information

• NCBI GI #: 4503899
• NCBI GeneID: 2588
• NCBI Accession #: NP_000503.1
• NCBI GenBank Nucleotide #: NM_000512.4
• UniProt Accession #: P34059; Q86VK3
• Molecular Weight: 58,026 Da
• NCBI Official Full Name: N-acetylgalactosamine-6-sulfatase
• NCBI Official Synonym Full Names: galactosamine (N-acetyl)-6-sulfatase
• NCBI Official Symbol: GALNS
• NCBI Official Synonym Symbols: GAS; MPS4A; GalN6S; GALNAC6S
• NCBI Protein Information: N-acetylgalactosamine-6-sulfatase
• UniProt Protein Name: N-acetylgalactosamine-6-sulfatase
• Protein Family: N-acetylgalactosamine-6-sulfatase
• UniProt Gene Name: GALNS
• UniProt Synonym Gene Names: Chondroitinase; GalN6S; GalNAc6S sulfatase
• UniProt Entry Name: GALNS_HUMAN

NCBI Description

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. [provided by RefSeq, Jul 2008]

Uniprot Description

GALNS: Defects in GALNS are the cause of mucopolysaccharidosis type 4A (MPS4A); also known as Morquio A syndrome. MPS4A is a form of mucopolysaccharidosis type 4, an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of keratan sulfate and chondroitin-6-sulfate. Key clinical features include short stature, skeletal dysplasia, dental anomalies, and corneal clouding. Intelligence is normal and there is no direct central nervous system involvement, although the skeletal changes may result in neurologic complications. There is variable severity, but patients with the severe phenotype usually do not survive past the second or third decade of life. Belongs to the sulfatase family.

Protein type: Hydrolase; EC 3.1.6.4; Glycan Metabolism - glycosaminoglycan degradation

Chromosomal Location of Human Ortholog: 16q24.3

Cellular Component: lysosomal lumen

Molecular Function: metal ion binding; N-acetylgalactosamine-4-sulfatase activity; N-acetylgalactosamine-6-sulfatase activity; sulfuric ester hydrolase activity

Biological Process: carbohydrate metabolic process; glycosaminoglycan metabolic process; keratan sulfate catabolic process; keratan sulfate metabolic process

Disease: Mucopolysaccharidosis, Type Iva