ELISA/assay

Human N-acetylgalactosamine-6-sulfatase, GALNS ELISA Kit

MBS2602718

48-Strip-Wells

275 USD

ELISA Kit

Human N-acetylgalactosamine-6-sulfatase, GALNS ELISA Kit

N-acetylgalactosamine-6-sulfatase, GALNS

N-acetylgalactosamine-6-sulfatase; N-acetylgalactosamine-6-sulfatase; N-acetylgalactosamine-6-sulfatase; chondroitinase; galNAc6S sulfatase; chondroitinsulfatase; galactose-6-sulfate sulfatase; N-acetylgalactosamine-6-sulfate sulfatase; galactosamine (N-acetyl)-6-sulfate sulfatase; galactosamine (N-acetyl)-6-sulfatase; Chondroitinsulfatase; Chondroitinase; Galactose-6-sulfate sulfatase; GalN6S; N-acetylgalactosamine-6-sulfate sulfatase; GalNAc6S sulfatase

GALNS

GALNS; GALNS; GAS; MPS4A; GalN6S; GALNAC6S; Chondroitinase; GalN6S; GalNAc6S sulfatase

GALNS_HUMAN

Human

No cross-reaction with other factors.

Store all reagents at 2-8 degree C.

Samples: Human serum, plasma or Cell Culture Supernatant and organizations in the natural and recombinant GALNS concentration. Assay Type: Sandwich. Detection Range: 20 ng/ml-0.312 ng/ml. Sensitivity: 0.06 ng/ml.

Intra-assay Precision: <= 8%. Inter-assay Precision: <= 12%

Priciple of the assay: This experiment use double-sandwich elisa technique and the ELISA Kit provided is typical. The pre-coated antibody is human GALNS monoclonal antibody and the detecting antibody is polyclonal antibody with biotin labeled. Samples and biotin labeling antibody are added into ELISA plate wells and washed out with PBS or TBS. Then Avidin-peroxidase conjugates are added to ELISA wells in order; Use TMB substrate for coloring after reactant thoroughly washed out by PBS or TBS. TMB turns into blue in peroxidase catalytic and finally turns into yellow under the action of acid. The color depth and the testing factors in samples are positively correlated.

4503899

NP_000503.1

NM_000512.4

P34059

58,026 Da

Chondroitin Sulfate Degradation Pathway (413376); Chondroitin Sulfate Degradation Pathway (468247); Glycosaminoglycan Degradation Pathway (82981); Glycosaminoglycan Degradation Pathway (355); Keratan Sulfate Degradation Pathway (413378); Keratan Sulfate Degradation Pathway (468269); Lysosome Pathway (99052); Lysosome Pathway (96865)

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]

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: Glycan Metabolism - glycosaminoglycan degradation; EC 3.1.6.4; Hydrolase. Chromosomal Location of Human Ortholog: 16q24.3. Cellular Component: lysosomal lumen. Molecular Function: N-acetylgalactosamine-4-sulfatase activity; N-acetylgalactosamine-6-sulfatase activity; metal ion binding; sulfuric ester hydrolase activity. Biological Process: keratan sulfate metabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis; keratan sulfate catabolic process. Disease: Mucopolysaccharidosis, Type Iva

48-Strip-Wells

275 USD

96-Strip-Wells

420