Keratan sulfate (KS)

Keratan sulfate (KS) is a sulfated glycosaminoglycan composed of repeating disaccharide units of galactose and N-acetylglucosamine. It is a vital component of the extracellular matrix, found in high concentrations in the cornea, cartilage, and bone, where it plays essential roles in maintaining tissue hydration and structural integrity. In the cornea, KS is critical for maintaining transparency, while in skeletal tissues, it provides mechanical stability and acts as a shock absorber in joints. Pathologically, the inability to degrade KS due to a deficiency in the enzyme N-acetylgalactosamine-6-sulfatase (GALNS) leads to Mucopolysaccharidosis type IVA (Morquio syndrome A), characterized by the toxic accumulation of KS in lysosomes and subsequent systemic skeletal dysplasia. Therapeutic strategies for such disorders involve enzyme replacement therapy, specifically Elosulfase alfa, which aims to reduce tissue accumulation by restoring the enzymatic degradation of KS. Additionally, KS levels in urine and blood serve as important biomarkers for diagnosing lysosomal storage diseases and monitoring the efficacy of therapeutic interventions.

Other names

KeratosulfateKeratan sulfate IKeratan sulfate IIKeratan sulfate IIIKS-IKS-IIKS-III

Enzyme replacement therapy (replaces deficient N-acetylgalactosamine-6-sulfatase to catalyze the hydrolytic cleavage of sulfate groups and degrade accumulated keratan sulfate)