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Código SKU: PKSH032823_50μg-ES
Proteína SGSH humana recombinante (etiqueta His), 50 μg
La N-sulfoglucosamina sulfohidrolasa (SGSH) es un miembro importante de la familia de las sulfatasas, que participa en la degradación del sulfato de heparina. La SGSH se une a un ion calcio por subunidad como cofactor. La SGSH cataliza la conversión de N-sulfo-D-glucosamina y H₂O en D-glucosamina y sulfato. La deficiencia de SGSH provoca la mucopolisacaridosis tipo 3A (MPS3A), una enfermedad recesiva por almacenamiento lisosomal que se caracteriza por disfunción neurológica, pero con manifestaciones somáticas relativamente leves.
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