FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2023136128> ?p ?o ?g. }

• W2023136128 endingPage "369" @default.
• W2023136128 startingPage "363" @default.
• W2023136128 abstract "Mucolipidosis (ML) II and III are rare autosomal recessively inherited diseases characterized by deficiency of multiple lysosomal enzymes and, as a result, a generalized storage of macromolecules in lysosomes of cells of mesenchymal origin. In ML II and ML III fibroblasts, most, but not all, newly synthesized lysosomal enzymes are secreted into the medium instead of being targeted correctly to lysosomes. Defects in the enzyme UDP-N-acetylglucosamine:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase underlie this effect. It is unknown how lysosomal phospholipases are targeted to the lysosomes of fibroblasts. In the present study lysosomal phospholipase activity was determined in delipidated fibroblast homogenates and plasma from ML II and ML III patients and controls using a [3H]choline-labeled phosphatidylcholine. After incubation, residual phosphatidylcholine and its labeled degradation products (lysophosphatidylcholine, glycerophosphorylcholine and choline phosphate) were quantified. We found that ML II and ML III fibroblasts are deficient in lysosomal phospholipase A and C activity. These enzymes were present in elevated amounts in plasma of ML II and ML III patients. These data indicate that phospholipases, like most other lysosomal enzymes in these diseases, are secreted into the blood instead of being targeted specifically to lysosomes. Thus, the mannose-6-phosphate receptor pathway is needed for proper delivery of lysosomal phospholipases to lysosomes. We also found that production of labeled choline phosphate was mainly due to the activity of acid sphingomyelinase instead of phospholipase C under the assay conditions used. Other active lipolytic enzymes were phospholipase A and lysophospholipase. No evidence for phospholipase D activity was found." @default.
• W2023136128 created "2016-06-24" @default.
• W2023136128 creator A5032149781 @default.
• W2023136128 creator A5072812826 @default.
• W2023136128 creator A5081431380 @default.
• W2023136128 date "1999-01-01" @default.
• W2023136128 modified "2023-09-25" @default.
• W2023136128 title "Lysosomal phospholipase activity is decreased in mucolipidosis II and III fibroblasts" @default.
• W2023136128 cites W1480165684 @default.
• W2023136128 cites W1491202632 @default.
• W2023136128 cites W1547423230 @default.
• W2023136128 cites W1559147139 @default.
• W2023136128 cites W1775749144 @default.
• W2023136128 cites W1967201541 @default.
• W2023136128 cites W1969392025 @default.
• W2023136128 cites W1972277479 @default.
• W2023136128 cites W1978530069 @default.
• W2023136128 cites W1978938824 @default.
• W2023136128 cites W1989434838 @default.
• W2023136128 cites W1992475281 @default.
• W2023136128 cites W1993947902 @default.
• W2023136128 cites W1996149231 @default.
• W2023136128 cites W2003222248 @default.
• W2023136128 cites W2014461574 @default.
• W2023136128 cites W2027648405 @default.
• W2023136128 cites W2031541160 @default.
• W2023136128 cites W2032276696 @default.
• W2023136128 cites W2044974423 @default.
• W2023136128 cites W2096650460 @default.
• W2023136128 cites W2112424491 @default.
• W2023136128 cites W2116543730 @default.
• W2023136128 cites W2125207129 @default.
• W2023136128 cites W2140596567 @default.
• W2023136128 cites W2168082981 @default.
• W2023136128 cites W2168526937 @default.
• W2023136128 cites W2321515281 @default.
• W2023136128 cites W2346207397 @default.
• W2023136128 cites W2416288664 @default.
• W2023136128 cites W2441007416 @default.
• W2023136128 cites W2475304766 @default.
• W2023136128 cites W299444643 @default.
• W2023136128 doi "https://doi.org/10.1016/s0005-2760(98)00129-5" @default.
• W2023136128 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/9989267" @default.
• W2023136128 hasPublicationYear "1999" @default.
• W2023136128 type Work @default.
• W2023136128 sameAs 2023136128 @default.
• W2023136128 citedByCount "5" @default.
• W2023136128 countsByYear W20231361282012 @default.
• W2023136128 crossrefType "journal-article" @default.
• W2023136128 hasAuthorship W2023136128A5032149781 @default.
• W2023136128 hasAuthorship W2023136128A5072812826 @default.
• W2023136128 hasAuthorship W2023136128A5081431380 @default.
• W2023136128 hasConcept C120694314 @default.
• W2023136128 hasConcept C12927208 @default.
• W2023136128 hasConcept C153911025 @default.
• W2023136128 hasConcept C180373561 @default.
• W2023136128 hasConcept C181199279 @default.
• W2023136128 hasConcept C185592680 @default.
• W2023136128 hasConcept C2776330855 @default.
• W2023136128 hasConcept C2778530336 @default.
• W2023136128 hasConcept C2778597717 @default.
• W2023136128 hasConcept C2778918659 @default.
• W2023136128 hasConcept C2780216420 @default.
• W2023136128 hasConcept C2780799628 @default.
• W2023136128 hasConcept C2780905385 @default.
• W2023136128 hasConcept C2781123555 @default.
• W2023136128 hasConcept C41625074 @default.
• W2023136128 hasConcept C55493867 @default.
• W2023136128 hasConcept C6182249 @default.
• W2023136128 hasConcept C86803240 @default.
• W2023136128 hasConceptScore W2023136128C120694314 @default.
• W2023136128 hasConceptScore W2023136128C12927208 @default.
• W2023136128 hasConceptScore W2023136128C153911025 @default.
• W2023136128 hasConceptScore W2023136128C180373561 @default.
• W2023136128 hasConceptScore W2023136128C181199279 @default.
• W2023136128 hasConceptScore W2023136128C185592680 @default.
• W2023136128 hasConceptScore W2023136128C2776330855 @default.
• W2023136128 hasConceptScore W2023136128C2778530336 @default.
• W2023136128 hasConceptScore W2023136128C2778597717 @default.
• W2023136128 hasConceptScore W2023136128C2778918659 @default.
• W2023136128 hasConceptScore W2023136128C2780216420 @default.
• W2023136128 hasConceptScore W2023136128C2780799628 @default.
• W2023136128 hasConceptScore W2023136128C2780905385 @default.
• W2023136128 hasConceptScore W2023136128C2781123555 @default.
• W2023136128 hasConceptScore W2023136128C41625074 @default.
• W2023136128 hasConceptScore W2023136128C55493867 @default.
• W2023136128 hasConceptScore W2023136128C6182249 @default.
• W2023136128 hasConceptScore W2023136128C86803240 @default.
• W2023136128 hasIssue "3" @default.
• W2023136128 hasLocation W20231361281 @default.
• W2023136128 hasLocation W20231361282 @default.
• W2023136128 hasOpenAccess W2023136128 @default.
• W2023136128 hasPrimaryLocation W20231361281 @default.
• W2023136128 hasRelatedWork W1540777723 @default.
• W2023136128 hasRelatedWork W163285947 @default.
• W2023136128 hasRelatedWork W1762755856 @default.
• W2023136128 hasRelatedWork W1978497443 @default.
• W2023136128 hasRelatedWork W1987606489 @default.

• next