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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

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

Kurt von Figura

Ljudmila V. Borissenko

Jens Fey

Jianhe Peng

Bernhard Schmidt

Thomas Dierks

Publisher:
Oxford University Press
DOI:10.1093/acprof:oso/9780198508786.003.0005

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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