N-Glycosylation Is Crucial for Folding, Trafficking, and Stability of Human Tripeptidyl-peptidase I
TLDR
A dual role of oligosaccharide at Asn-286 in folding and lysosomal targeting could contribute to the unusual, but cell type-dependent, fate of misfolded TPP I conformer and represent the molecular basis of the disease process in subjects with naturally occurring missense mutation.About:
This article is published in Journal of Biological Chemistry.The article was published on 2004-03-26 and is currently open access. It has received 98 citations till now. The article focuses on the topics: Tripeptidyl peptidase I & N-linked glycosylation.read more
Citations
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Effects of glycosylation on the stability of protein pharmaceuticals
Ricardo J. Solá,Kai Griebenow +1 more
TL;DR: A survey of the different instabilities displayed by protein pharmaceuticals is presented, by addressing which of these instabilities can be improved by glycosylation, and by discussing the possible mechanisms by which glycans induce these stabilization effects.
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Neuronal ceroid lipofuscinoses
Anu Jalanko,Thomas Braulke +1 more
TL;DR: The current knowledge of the NCL proteins, basic characteristics of each disease and studies of pathogenetic mechanisms in animal models of these diseases are summarized.
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N-linked oligosaccharides as outfitters for glycoprotein folding, form and function
TL;DR: It is evident that glycosylation is a multifaceted post-translational modification that serves to "outfit" proteins for fold-function balance.
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Correlations between genotype, ultrastructural morphology and clinical phenotype in the neuronal ceroid lipofuscinoses.
TL;DR: This review attempts to correlate the gene, disease-causing mutation, morphology and clinical phenotype for each type of NCL.
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Controlling N-linked glycan site occupancy.
TL;DR: The characterization of the dolichol pyrophosphate biosynthetic pathway and the recent identification of potential rate limiting enzymes in yeast and mammalian cells has made it possible to investigate their role in site occupancy, and insights into the location and residues in and around the acceptor tripeptide sequon suggest an influence on N-glycan site occupancy.
References
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Site-directed mutagenesis by overlap extension using the polymerase chain reaction.
TL;DR: In this paper, complementary oligodeoxyribonucleotide (oligo) primers and the polymerase chain reaction are used to generate two DNA fragments having overlapping ends, and these fragments are combined in a subsequent 'fusion' reaction in which the overlapping ends anneal, allowing the 3' overlap of each strand to serve as a primer for the three' extension of the complementary strand.
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Quality control in the endoplasmic reticulum
Lars Ellgaard,Ari Helenius +1 more
TL;DR: Recent progress is discussed in understanding the conformation-specific sorting of proteins at the level of ER retention and export, which is important for the fidelity of cellular functions.
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The Biogenesis of Lysosomes
Stuart Kornfeld,Ira Mellman +1 more
TL;DR: Article de synthese sur les lysosomes: biogenese, definition, role biologique, Les enzymes lysOSomales sont decrites ainsi that leur role dans l'exo et l'endocytose.
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Orchestrating the unfolded protein response in health and disease
TL;DR: As the authors gain a greater understanding of the mechanisms that control UPR activation, it should be possible to discover methods to activate or inhibit the UPR as desired for therapeutic benefit.
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Structure and function of the mannose 6-phosphate/insulinlike growth factor II receptors.
TL;DR: The role of Receptor Trafficking in Cell Extracts, Lysosomal Enzyme Sorting and Endocytosis, and Role in Signal Transduction are highlighted.