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A defect in β-galactosidase of B lymphocytes in the osteopetrotic (opop) mouse

✍ Scribed by Nobuto Yamamoto; Venkateswara R. Naraparaju

Book ID
107908624
Publisher
Elsevier Science
Year
1996
Tongue
English
Weight
632 KB
Volume
50
Category
Article
ISSN
0165-2478

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Morquio B syndrome: A primary defect in
Morquio B syndrome: A primary defect in β-galactosidase
✍ van der Horst, Gijsbertus T. J. ;Kleijer, Wim J. ;Hoogeveen, André T. ;Huijmans, 📂 Article 📅 1983 🏛 John Wiley and Sons 🌐 English ⚖ 902 KB

Fibroblasts from patients with Morquio B syndrome contain normal numbers of 0-galactosidase molecules with normal turnover but strongly reduced activity per enzyme molecule. Various substrate affinities are abnormal: the K, for methylum belliferyl (MU)-P-galactoside is 4-10-fold elevated and affinit