Developmental Changes in the Expression of Neuronal Ceroid Lipofuscinoses-Linked Proteins

Neuronal ceroid lipofuscinoses (NCL) form a distinct group of storage diseases where the normal development of the central nervous system is interrupted and neurons of the neocortex begin to degenerate. Mutations in genes encoding three lysosomal enzymes are the causes for three early-onset forms of...

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Veröffentlicht in:	Molecular Genetics and Metabolism 2000-09, Vol.71 (1-2), p.190-194
Hauptverfasser:	Suopanki, Jaana, Partanen, Sanna, Ezaki, Junji, Baumann, Marc, Kominami, Eiki, Tyynelä, Jaana
Format:	Artikel
Sprache:	eng
Schlagworte:	Aminopeptidases Animals Base Sequence cathepsin D Cathepsin D - genetics Cathepsin D - metabolism Central Nervous System - enzymology Central Nervous System - growth & development Central Nervous System - pathology developmental expression Dipeptidyl-Peptidases and Tripeptidyl-Peptidases DNA Primers - genetics Endopeptidases - genetics Endopeptidases - metabolism Gene Expression Regulation, Developmental Humans lysosomes Lysosomes - enzymology Mutation Nerve Degeneration neurodegeneration neuronal ceroid lipofuscinosis Neuronal Ceroid-Lipofuscinoses - enzymology Neuronal Ceroid-Lipofuscinoses - genetics Neuronal Ceroid-Lipofuscinoses - pathology palmitoyl-protein thioesterase 1 Rats RNA, Messenger - genetics RNA, Messenger - metabolism Serine Proteases Thiolester Hydrolases - genetics Thiolester Hydrolases - metabolism tripeptidyl peptidase 1
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