Gpnmb Is a Potential Marker for the Visceral Pathology in Niemann-Pick Type C Disease

Impaired function of NPC1 or NPC2 lysosomal proteins leads to the intracellular accumulation of unesterified cholesterol, the primary defect underlying Niemann-Pick type C (NPC) disease. In addition, glycosphingolipids (GSLs) accumulate in lysosomes as well. Intralysosomal lipid accumulation trigger...

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Veröffentlicht in:	PloS one Jg. 11; H. 1; S. e0147208
Hauptverfasser:	Marques, André R. A., Gabriel, Tanit L., Aten, Jan, van Roomen, Cindy P. A. A., Ottenhoff, Roelof, Claessen, Nike, Alfonso, Pilar, Irún, Pilar, Giraldo, Pilar, Aerts, Johannes M. F. G., van Eijk, Marco
Format:	Journal Article
Sprache:	Englisch
Veröffentlicht:	United States Public Library of Science 15.01.2016 Public Library of Science (PLoS)
Schlagworte:	Accumulation Adult Aged Aged, 80 and over Animal tissues Animals Biochemistry Bioindicators Biomarkers Biomarkers - metabolism Brain diseases Care and treatment Cell Line Ceramide glucosyltransferase Cholesterol Cholesterol - metabolism Development and progression Disease Models, Animal Eye Proteins - genetics Eye Proteins - metabolism Female Foam Cells - metabolism Genetic disorders Glycoproteins Glycosphingolipids Glycosphingolipids - metabolism Humans Inborn errors of metabolism Laboratories Lipids Liver Lysosomes Macrophages Male Melanoma Membrane Glycoproteins - genetics Membrane Glycoproteins - metabolism Metabolic disorders Metabolism Mice Middle Aged Mucopolysaccharides Niemann-Pick disease Niemann-Pick Disease, Type C - genetics Niemann-Pick Disease, Type C - metabolism Npc1 protein Pathology Patient outcomes Proteins Risk factors Rodents Secretion Transcription Viscera Yang, Cindy Young Adult United States Netherlands Spain
ISSN:	1932-6203, 1932-6203
Online-Zugang:	Volltext
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