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Various substrate affinities are abnormal: the K<jats:sub>M<\/jats:sub> for methylum belliferyl (MU)\u2010\u03b2\u2010galactoside is 4\u201310\u2010fold elevated and affinity for keratan sulphate and oligosaccharides, isolated from Morquio B urine, was not detectable. In contrast, these substrate affinities are normal for \u03b2\u2010galactosidase in adult type G<jats:sub>M1<\/jats:sub>\u2010gangliosidosis fibroblasts. Cell hybridization studies demonstrate that Morquio B syndrome and infantile and adult type G<jats:sub>M1<\/jats:sub>\u2010gangliosidosis belong to the same complementation group. From these results we conclude that Morquio B syndrome is caused by a mutation in the structural gene for \u03b2\u2010galactosidase, which is allelic to the mutations in infantile and adult type G<jats:sub>M1<\/jats:sub>\u2010gangliosidosis. Urinary excretion of keratan sulphate and oligosaccharides is abnormal in Morquio B syndrome but normal in adult type G<jats:sub>M1<\/jats:sub>\u2010gangliosidosis. The catalytic properties of \u03b2\u2010galactosidase in Morquio B syndrome and G<jats:sub>M1<\/jats:sub>\u2010gangliosidosis provide a possible explanation for the distinct clinical manifestations in these disorders.<\/jats:p>","DOI":"10.1002\/ajmg.1320160215","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T18:59:12Z","timestamp":1118516352000},"page":"261-275","source":"Crossref","is-referenced-by-count":43,"title":["Morquio B syndrome: A primary defect in \u03b2\u2010galactosidase"],"prefix":"10.1002","volume":"16","author":[{"given":"Gijsbertus T. 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