Matches in SemOpenAlex for { <https://semopenalex.org/work/W2087786593> ?p ?o ?g. }
Showing items 1 to 96 of
96
with 100 items per page.
- W2087786593 endingPage "56" @default.
- W2087786593 startingPage "49" @default.
- W2087786593 abstract "Biotinidase deficiency is an autosomal recessively inherited metabolic disorder that can be easily and effectively treated with pharmacological doses of the vitamin, biotin. Untreated children with profound biotinidase deficiency may exhibit neurological, cutaneous and cellular immunological abnormalities, specifically candida infections. To better understand the immunological dysfunction in some symptomatic individuals with biotinidase deficiency, we studied various aspects of immunological function in a genetically engineered knock-out mouse with biotinidase deficiency. The mouse has no detectable biotinidase activity and develops neurological and cutaneous symptoms similar to those seen in symptomatic children with the disorder. Mice with profound biotinidase deficiency on a biotin-restricted diet had smaller thymuses and spleens than identical mice fed a biotin-replete diet or wildtype mice on either diet; however, the organ to body weight ratios were not significantly different. Thymus histology was normal. Splenocyte subpopulation study showed a significant increase in CD4 positive cells. In addition, in vitro lymphocyte proliferation assays consistently showed diminished proliferation in response to various immunological stimuli. Not all symptomatic individuals with profound biotinidase deficiency develop immunological dysfunction; however, our results do show significant alterations in cellular immunological function that may contribute and/or provide a mechanism(s) for the cellular immunity abnormalities in individuals with biotinidase deficiency." @default.
- W2087786593 created "2016-06-24" @default.
- W2087786593 creator A5011283569 @default.
- W2087786593 creator A5014068004 @default.
- W2087786593 creator A5044126197 @default.
- W2087786593 creator A5074413749 @default.
- W2087786593 date "2014-05-01" @default.
- W2087786593 modified "2023-10-12" @default.
- W2087786593 title "Characterization and functional analysis of cellular immunity in mice with biotinidase deficiency" @default.
- W2087786593 cites W1972509889 @default.
- W2087786593 cites W1986898728 @default.
- W2087786593 cites W1989445118 @default.
- W2087786593 cites W1995422140 @default.
- W2087786593 cites W2000441830 @default.
- W2087786593 cites W2014819128 @default.
- W2087786593 cites W2018791025 @default.
- W2087786593 cites W2020103041 @default.
- W2087786593 cites W2022195677 @default.
- W2087786593 cites W2036849145 @default.
- W2087786593 cites W2059092049 @default.
- W2087786593 cites W2060975471 @default.
- W2087786593 cites W2067146840 @default.
- W2087786593 cites W2070379407 @default.
- W2087786593 cites W2097366516 @default.
- W2087786593 cites W2104453235 @default.
- W2087786593 cites W2110048959 @default.
- W2087786593 cites W2114918609 @default.
- W2087786593 cites W2123229213 @default.
- W2087786593 cites W2125311191 @default.
- W2087786593 cites W2158485667 @default.
- W2087786593 cites W2265130166 @default.
- W2087786593 cites W2340979936 @default.
- W2087786593 cites W3021646946 @default.
- W2087786593 doi "https://doi.org/10.1016/j.ymgme.2014.02.008" @default.
- W2087786593 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/24630269" @default.
- W2087786593 hasPublicationYear "2014" @default.
- W2087786593 type Work @default.
- W2087786593 sameAs 2087786593 @default.
- W2087786593 citedByCount "10" @default.
- W2087786593 countsByYear W20877865932015 @default.
- W2087786593 countsByYear W20877865932016 @default.
- W2087786593 countsByYear W20877865932020 @default.
- W2087786593 countsByYear W20877865932021 @default.
- W2087786593 countsByYear W20877865932023 @default.
- W2087786593 crossrefType "journal-article" @default.
- W2087786593 hasAuthorship W2087786593A5011283569 @default.
- W2087786593 hasAuthorship W2087786593A5014068004 @default.
- W2087786593 hasAuthorship W2087786593A5044126197 @default.
- W2087786593 hasAuthorship W2087786593A5074413749 @default.
- W2087786593 hasConcept C126322002 @default.
- W2087786593 hasConcept C134018914 @default.
- W2087786593 hasConcept C203014093 @default.
- W2087786593 hasConcept C2776294945 @default.
- W2087786593 hasConcept C2778038050 @default.
- W2087786593 hasConcept C2778204606 @default.
- W2087786593 hasConcept C2779341262 @default.
- W2087786593 hasConcept C3017875465 @default.
- W2087786593 hasConcept C55493867 @default.
- W2087786593 hasConcept C71924100 @default.
- W2087786593 hasConcept C86803240 @default.
- W2087786593 hasConcept C8891405 @default.
- W2087786593 hasConceptScore W2087786593C126322002 @default.
- W2087786593 hasConceptScore W2087786593C134018914 @default.
- W2087786593 hasConceptScore W2087786593C203014093 @default.
- W2087786593 hasConceptScore W2087786593C2776294945 @default.
- W2087786593 hasConceptScore W2087786593C2778038050 @default.
- W2087786593 hasConceptScore W2087786593C2778204606 @default.
- W2087786593 hasConceptScore W2087786593C2779341262 @default.
- W2087786593 hasConceptScore W2087786593C3017875465 @default.
- W2087786593 hasConceptScore W2087786593C55493867 @default.
- W2087786593 hasConceptScore W2087786593C71924100 @default.
- W2087786593 hasConceptScore W2087786593C86803240 @default.
- W2087786593 hasConceptScore W2087786593C8891405 @default.
- W2087786593 hasFunder F4320306230 @default.
- W2087786593 hasIssue "1" @default.
- W2087786593 hasLocation W20877865931 @default.
- W2087786593 hasLocation W20877865932 @default.
- W2087786593 hasOpenAccess W2087786593 @default.
- W2087786593 hasPrimaryLocation W20877865931 @default.
- W2087786593 hasRelatedWork W2027622304 @default.
- W2087786593 hasRelatedWork W2117757471 @default.
- W2087786593 hasRelatedWork W2152260884 @default.
- W2087786593 hasRelatedWork W2160133043 @default.
- W2087786593 hasRelatedWork W2265125857 @default.
- W2087786593 hasRelatedWork W2339159816 @default.
- W2087786593 hasRelatedWork W2407256926 @default.
- W2087786593 hasRelatedWork W3032210197 @default.
- W2087786593 hasRelatedWork W4283821855 @default.
- W2087786593 hasRelatedWork W4381570565 @default.
- W2087786593 hasVolume "112" @default.
- W2087786593 isParatext "false" @default.
- W2087786593 isRetracted "false" @default.
- W2087786593 magId "2087786593" @default.
- W2087786593 workType "article" @default.