Matches in SemOpenAlex for { <https://semopenalex.org/work/W2039563731> ?p ?o ?g. }
Showing items 1 to 98 of
98
with 100 items per page.
- W2039563731 endingPage "167" @default.
- W2039563731 startingPage "156" @default.
- W2039563731 abstract "Enzyme replacement is a potential therapy for mucopolysaccharidosis I (MPS I), a lysosomal storage disorder caused by α-L-iduronidase deficiency. Previous work showed improvement in the tissues of MPS I dogs treated intravenously for 3 months with recombinant human α-L-iduronidase (25,000 units or ∼0.1 mg/kg/week). We have now treated an MPS I-affected dog for 13 months to assess the clinical effects of enzyme replacement. The treated dog gained more weight, was more active, and had less joint stiffness than the untreated littermate. Biochemical and histologic studies demonstrated uptake of α-L-iduronidase and decreased lysosomal storage in the liver, kidney, spleen, lymph nodes, synovium, adrenals, and lungs. The brain had detectable enzyme activity and decreased glycosaminoglycan storage although histologic improvement was not evident. Cartilage and heart valve did not show any detectable improvement. A fivefold higher dose (∼0.5 mg/kg) administered five times over 10 days to two other dogs resulted in higher tissue enzyme activity and similarly decreased glycosaminoglycan storage and excretion. Antibodies to human α-L-iduronidase were induced in all treated dogs and may be associated with immune complex deposition and proteinuria. Recombinant canine α-L-iduronidase also induced antibody formation to a similar degree. The results support the conclusion that enzyme replacement is a promising therapy for MPS I though immunologic complications may occur." @default.
- W2039563731 created "2016-06-24" @default.
- W2039563731 creator A5001228618 @default.
- W2039563731 creator A5003323119 @default.
- W2039563731 creator A5006551650 @default.
- W2039563731 creator A5021076432 @default.
- W2039563731 creator A5031070002 @default.
- W2039563731 creator A5064239905 @default.
- W2039563731 creator A5068463703 @default.
- W2039563731 creator A5074066164 @default.
- W2039563731 creator A5078475852 @default.
- W2039563731 creator A5082123381 @default.
- W2039563731 date "1996-08-01" @default.
- W2039563731 modified "2023-10-18" @default.
- W2039563731 title "Long-Term and High-Dose Trials of Enzyme Replacement Therapy in the Canine Model of Mucopolysaccharidosis I" @default.
- W2039563731 doi "https://doi.org/10.1006/bmme.1996.0044" @default.
- W2039563731 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/8812735" @default.
- W2039563731 hasPublicationYear "1996" @default.
- W2039563731 type Work @default.
- W2039563731 sameAs 2039563731 @default.
- W2039563731 citedByCount "162" @default.
- W2039563731 countsByYear W20395637312012 @default.
- W2039563731 countsByYear W20395637312013 @default.
- W2039563731 countsByYear W20395637312014 @default.
- W2039563731 countsByYear W20395637312015 @default.
- W2039563731 countsByYear W20395637312016 @default.
- W2039563731 countsByYear W20395637312017 @default.
- W2039563731 countsByYear W20395637312018 @default.
- W2039563731 countsByYear W20395637312019 @default.
- W2039563731 countsByYear W20395637312020 @default.
- W2039563731 countsByYear W20395637312021 @default.
- W2039563731 countsByYear W20395637312022 @default.
- W2039563731 countsByYear W20395637312023 @default.
- W2039563731 crossrefType "journal-article" @default.
- W2039563731 hasAuthorship W2039563731A5001228618 @default.
- W2039563731 hasAuthorship W2039563731A5003323119 @default.
- W2039563731 hasAuthorship W2039563731A5006551650 @default.
- W2039563731 hasAuthorship W2039563731A5021076432 @default.
- W2039563731 hasAuthorship W2039563731A5031070002 @default.
- W2039563731 hasAuthorship W2039563731A5064239905 @default.
- W2039563731 hasAuthorship W2039563731A5068463703 @default.
- W2039563731 hasAuthorship W2039563731A5074066164 @default.
- W2039563731 hasAuthorship W2039563731A5078475852 @default.
- W2039563731 hasAuthorship W2039563731A5082123381 @default.
- W2039563731 hasConcept C105702510 @default.
- W2039563731 hasConcept C126322002 @default.
- W2039563731 hasConcept C134018914 @default.
- W2039563731 hasConcept C153074725 @default.
- W2039563731 hasConcept C181199279 @default.
- W2039563731 hasConcept C185592680 @default.
- W2039563731 hasConcept C2778264360 @default.
- W2039563731 hasConcept C2779134260 @default.
- W2039563731 hasConcept C2779416428 @default.
- W2039563731 hasConcept C2779969927 @default.
- W2039563731 hasConcept C2780091579 @default.
- W2039563731 hasConcept C2780931953 @default.
- W2039563731 hasConcept C2910509766 @default.
- W2039563731 hasConcept C2910727661 @default.
- W2039563731 hasConcept C55493867 @default.
- W2039563731 hasConcept C71924100 @default.
- W2039563731 hasConceptScore W2039563731C105702510 @default.
- W2039563731 hasConceptScore W2039563731C126322002 @default.
- W2039563731 hasConceptScore W2039563731C134018914 @default.
- W2039563731 hasConceptScore W2039563731C153074725 @default.
- W2039563731 hasConceptScore W2039563731C181199279 @default.
- W2039563731 hasConceptScore W2039563731C185592680 @default.
- W2039563731 hasConceptScore W2039563731C2778264360 @default.
- W2039563731 hasConceptScore W2039563731C2779134260 @default.
- W2039563731 hasConceptScore W2039563731C2779416428 @default.
- W2039563731 hasConceptScore W2039563731C2779969927 @default.
- W2039563731 hasConceptScore W2039563731C2780091579 @default.
- W2039563731 hasConceptScore W2039563731C2780931953 @default.
- W2039563731 hasConceptScore W2039563731C2910509766 @default.
- W2039563731 hasConceptScore W2039563731C2910727661 @default.
- W2039563731 hasConceptScore W2039563731C55493867 @default.
- W2039563731 hasConceptScore W2039563731C71924100 @default.
- W2039563731 hasIssue "2" @default.
- W2039563731 hasLocation W20395637311 @default.
- W2039563731 hasLocation W20395637312 @default.
- W2039563731 hasOpenAccess W2039563731 @default.
- W2039563731 hasPrimaryLocation W20395637311 @default.
- W2039563731 hasRelatedWork W2039563731 @default.
- W2039563731 hasRelatedWork W2093627367 @default.
- W2039563731 hasRelatedWork W2101631364 @default.
- W2039563731 hasRelatedWork W2321043350 @default.
- W2039563731 hasRelatedWork W2327110361 @default.
- W2039563731 hasRelatedWork W2604371438 @default.
- W2039563731 hasRelatedWork W3128228216 @default.
- W2039563731 hasRelatedWork W4226453154 @default.
- W2039563731 hasRelatedWork W4308256952 @default.
- W2039563731 hasRelatedWork W2225131080 @default.
- W2039563731 hasVolume "58" @default.
- W2039563731 isParatext "false" @default.
- W2039563731 isRetracted "false" @default.
- W2039563731 magId "2039563731" @default.
- W2039563731 workType "article" @default.