Anti-GALNS/Chondroitinase antibody

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Chondroitinase also known as N-acetylgalactosamine-6-sulfatase (GALNS) is an enzyme involved in the degradation of glycosaminoglycans such as chondroitin sulfate and keratan sulfate. It is a lysosomal hydrolase enzyme that plays an important role in remodeling the extracellular matrix by cleaving sulfate groups from these molecules. The molecular mass of GALNS is approximately 60 kDa. GALNS expression primarily occurs in tissues like cartilage bones and connective tissues reflecting its function in skeletal development and maintenance.

Biological function summary

N-acetylgalactosamine-6-sulfatase acts as a critical player in catabolism breaking down complex polysaccharides in lysosomes. By degrading glycosaminoglycans it prevents accumulation of excessive polysaccharides in cells which might lead to cellular dysfunction. GALNS operates as a single molecule not forming any known complexes yet its activity is integral to maintaining cellular homeostasis and preventing pathological buildup of substrates within cells.

Pathways

GALNS functions in the glycosaminoglycan metabolism pathway essential for recycling cellular materials from the extracellular matrix. Another related pathway is the lysosomal degradation pathway where it relates to other enzymes like iduronate-2-sulfatase that also participate in the breakdown of complex molecules. These pathways collectively help in maintaining normal cellular function by facilitating the turnover of matrix components.

Associated diseases and disorders

N-acetylgalactosamine-6-sulfatase relates to Morquio A syndrome a lysosomal storage disorder caused by deficiencies in GALNS activity. This leads to abnormal accumulation of keratan sulfate and chondroitin sulfate resulting in skeletal abnormalities and growth issues. GALNS has also connections to mucopolysaccharidosis IV where defects in this enzyme similarly cause buildup of glycosaminoglycans. Proteins like N-sulfoglucosamine sulfohydrolase show association with GALNS through shared pathways in these disorders highlighting the interconnected nature of enzyme functions in lysosomal storage diseases.