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Lysosomal Disorders of the BrainRecent Advances in Molecular and Cellular Pathogenesis and Treatment$
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Frances Platt and Steven Walkley

Print publication date: 2004

Print ISBN-13: 9780198508786

Published to Oxford Scholarship Online: September 2009

DOI: 10.1093/acprof:oso/9780198508786.001.0001

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Defects in lysosomal enzyme modification for catalytic activity

Defects in lysosomal enzyme modification for catalytic activity

(p.131) Chapter 5 Defects in lysosomal enzyme modification for catalytic activity
Lysosomal Disorders of the Brain

Kurt von Figura

Ljudmila V. Borissenko

Jens Fey

Jianhe Peng

Bernhard Schmidt

Thomas Dierks

Oxford University Press

Two types of modifications are known so far that are required for catalytic activity of lysosomal enzymes. The first type represents the conversion of the catalytically inactive pro-form of cysteinyl- and aspartyl-proteinases into the catalytically active mature form by limited proteolysis. This chapter focuses on the second type of modification, which is represented by the posttranslational generation of a Ca-formylglycine (FGly) residue in the catalytic centre of sulfatases. Deficiency of this modification is the molecular cause of multiple sulfatase deficiency (MSD).

Keywords:   lysosomal enzymes, multiple sulfatase deficiency, Ca-formylglycine residue, sulfatases

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