Regulation of the lysosome-associated membrane protein in a sucrose model of lysosomal storage

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dc.contributor.authorIsaac, E.
dc.contributor.authorKarageorgos, L.
dc.contributor.authorBrooks, D.
dc.contributor.authorHopwood, J.
dc.contributor.authorMeikle, P.
dc.date.issued2000
dc.description.abstractLysosomal biogenesis is a complex process requiring the coordinated expression and colocalization of numerous soluble and membrane proteins. In storage disorders, lysosomal biogenesis is regulated at least partially at, or prior to, the level of mRNA. We have used the sucrosome storage model to determine the sites of regulation of LAMP-1, a major constituent of the lysosomal membrane. A six- to eightfold increase in the level of LAMP-1 mRNA and protein was observed in response to sucrose storage. The half-life of LAMP-1 mRNA was not significantly different in cells grown in the absence or presence of sucrose, implying that the increase observed in mRNA levels reflects an increase in the rate of transcription. The sixfold increase in mRNA did not translate into an increase in LAMP-1 synthesis, indicating an overall decrease in the translational yield in sucrosome cells. The elevation of LAMP-1 protein levels in storage cells was due in large part to a threefold increase in the half-life of the protein. These results are discussed in view of the current understanding of lysosomal biogenesis and how this process is altered during storage.
dc.identifier.citationExperimental Cell Research, 2000; 254(2):204-209
dc.identifier.doi10.1006/excr.1999.4755
dc.identifier.issn0014-4827
dc.identifier.issn1090-2422
dc.identifier.orcidBrooks, D. [0000-0001-9098-3626]
dc.identifier.urihttp://hdl.handle.net/2440/7234
dc.language.isoen
dc.publisherAcademic Press Inc Elsevier Science
dc.source.urihttps://doi.org/10.1006/excr.1999.4755
dc.subjectCell Line
dc.subjectCHO Cells
dc.subjectLysosomes
dc.subjectFibroblasts
dc.subjectSkin
dc.subjectAnimals
dc.subjectHumans
dc.subjectLysosomal Storage Diseases
dc.subjectSucrose
dc.subjectMembrane Glycoproteins
dc.subjectRecombinant Proteins
dc.subjectRNA, Messenger
dc.subjectAntigens, CD
dc.subjectTransfection
dc.subjectTranscription, Genetic
dc.subjectCricetinae
dc.subjectLysosomal Membrane Proteins
dc.titleRegulation of the lysosome-associated membrane protein in a sucrose model of lysosomal storage
dc.typeJournal article
pubs.publication-statusPublished

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