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Intracellular trafficking of lysosomal proteins and lysosomes
Int. Journal of Clinical Pharmacology and Therapeutics, 2009In the synthesis and trafficking of precursors of most lysosomal matrix proteins, the stages necessary for lysosomal delivery include the addition of phosphorylated mannose-rich oligosaccharides, binding of the modified proteins to receptors, their segregation from the secretory pathways and delivery to the endosomal pathway.
A, Hasilik, C, Wrocklage, B, Schröder
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2022
Lysosomal storage diseases (LSDs) are a group of inherited metabolic disorders that result from lysosomal dysfunction. Most LSDs are autosomal recessively inherited disorders, with only three exceptions that are X-linked. The overall incidence is about 1 in 5000 live births.
Funghini, Silvia +3 more
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Lysosomal storage diseases (LSDs) are a group of inherited metabolic disorders that result from lysosomal dysfunction. Most LSDs are autosomal recessively inherited disorders, with only three exceptions that are X-linked. The overall incidence is about 1 in 5000 live births.
Funghini, Silvia +3 more
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[Lysosomes and lysosomal storage diseases].
Journal de la Societe de biologie, 2002Lysosomal storage disorders (LSDs) are monogenic inborn errors of metabolism. Various groups have been delineated according to the affected pathway and the accumulated substrate, and new entities are still being identified. They are severe disorders with a heterogeneous clinical spectrum encompassing visceral, skeletal and neurologic involvement, and ...
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