FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2155987029> ?p ?o ?g. }

• W2155987029 abstract "The neuronal ceroid lipofuscinoses constitute a group of fatal inherited lysosomal storage diseases that manifest in profound neurodegeneration in the CNS. Visual impairment usually is an early symptom and selective degeneration of retinal neurons has been described in patients suffering from distinct disease subtypes. We have previously demonstrated that palmitoyl protein thioesterase 1 deficient (Ppt1 -/- ) mice, a model of the infantile disease subtype, exhibit progressive axonal degeneration in the optic nerve and loss of retinal ganglion cells, faithfully reflecting disease severity in the CNS. Here we performed spectral domain optical coherence tomography (OCT) in Ppt1 -/- and ceroid lipofuscinosis neuronal 3 deficient (Cln3 -/- ) mice, which are models of infantile and juvenile neuronal ceroid lipofuscinosis, respectively, in order to establish a non-invasive method to assess retinal alterations and monitor disease severity in vivo. Blue laser autofluorescence imaging revealed increased accumulation of autofluorescent storage material in the inner retinae of 7-month-old Ppt1 -/- and of 16-month-old Cln3 -/- mice in comparison with age-matched control littermates. Additionally, optical coherence tomography demonstrated reduced thickness of retinae in knockout mice in comparison with age-matched control littermates. High resolution scans and manual measurements allowed for separation of different retinal composite layers and revealed a thinning of layers in the inner retinae of both mouse models at distinct ages. OCT measurements correlated well with subsequent histological analysis of the same retinae. These results demonstrate the feasibility of OCT to assess neurodegenerative disease severity in mouse models of neuronal ceroid lipofuscinosis and might have important implications for diagnostic evaluation of disease progression and therapeutic efficacy in patients. Moreover, the non-invasive method allows for longitudinal studies in experimental models, reducing the number of animals used for research." @default.
• W2155987029 created "2016-06-24" @default.
• W2155987029 creator A5018543083 @default.
• W2155987029 creator A5038033677 @default.
• W2155987029 creator A5046874780 @default.
• W2155987029 creator A5057863942 @default.
• W2155987029 date "2014-05-10" @default.
• W2155987029 modified "2023-10-05" @default.
• W2155987029 title "Non-invasive assessment of retinal alterations in mouse models of infantile and juvenile neuronal ceroid lipofuscinosis by spectral domain optical coherence tomography" @default.
• W2155987029 cites W1970789407 @default.
• W2155987029 cites W1983641273 @default.
• W2155987029 cites W1988967065 @default.
• W2155987029 cites W2002534526 @default.
• W2155987029 cites W2013037534 @default.
• W2155987029 cites W2014641941 @default.
• W2155987029 cites W2022891079 @default.
• W2155987029 cites W2025707873 @default.
• W2155987029 cites W2028246239 @default.
• W2155987029 cites W2038812410 @default.
• W2155987029 cites W2044670556 @default.
• W2155987029 cites W2047162457 @default.
• W2155987029 cites W2047774213 @default.
• W2155987029 cites W2052979981 @default.
• W2155987029 cites W2058030141 @default.
• W2155987029 cites W2058078854 @default.
• W2155987029 cites W2059760539 @default.
• W2155987029 cites W2059906626 @default.
• W2155987029 cites W2072573175 @default.
• W2155987029 cites W2073337321 @default.
• W2155987029 cites W2082497158 @default.
• W2155987029 cites W2086080658 @default.
• W2155987029 cites W2087681061 @default.
• W2155987029 cites W2098998600 @default.
• W2155987029 cites W2104301336 @default.
• W2155987029 cites W2105732944 @default.
• W2155987029 cites W2113922996 @default.
• W2155987029 cites W2124523908 @default.
• W2155987029 cites W2125574371 @default.
• W2155987029 cites W2126213759 @default.
• W2155987029 cites W2133628800 @default.
• W2155987029 cites W2141010238 @default.
• W2155987029 cites W2141273703 @default.
• W2155987029 cites W2144968601 @default.
• W2155987029 cites W2171116763 @default.
• W2155987029 cites W255710148 @default.
• W2155987029 doi "https://doi.org/10.1186/2051-5960-2-54" @default.
• W2155987029 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/4035096" @default.
• W2155987029 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/24887158" @default.
• W2155987029 hasPublicationYear "2014" @default.
• W2155987029 type Work @default.
• W2155987029 sameAs 2155987029 @default.
• W2155987029 citedByCount "33" @default.
• W2155987029 countsByYear W21559870292015 @default.
• W2155987029 countsByYear W21559870292016 @default.
• W2155987029 countsByYear W21559870292017 @default.
• W2155987029 countsByYear W21559870292018 @default.
• W2155987029 countsByYear W21559870292020 @default.
• W2155987029 countsByYear W21559870292021 @default.
• W2155987029 countsByYear W21559870292022 @default.
• W2155987029 countsByYear W21559870292023 @default.
• W2155987029 crossrefType "journal-article" @default.
• W2155987029 hasAuthorship W2155987029A5018543083 @default.
• W2155987029 hasAuthorship W2155987029A5038033677 @default.
• W2155987029 hasAuthorship W2155987029A5046874780 @default.
• W2155987029 hasAuthorship W2155987029A5057863942 @default.
• W2155987029 hasBestOaLocation W21559870291 @default.
• W2155987029 hasConcept C118487528 @default.
• W2155987029 hasConcept C142724271 @default.
• W2155987029 hasConcept C169760540 @default.
• W2155987029 hasConcept C186198217 @default.
• W2155987029 hasConcept C2776925932 @default.
• W2155987029 hasConcept C2777093970 @default.
• W2155987029 hasConcept C2778230176 @default.
• W2155987029 hasConcept C2778650512 @default.
• W2155987029 hasConcept C2778818243 @default.
• W2155987029 hasConcept C2779134260 @default.
• W2155987029 hasConcept C2780827179 @default.
• W2155987029 hasConcept C2781040256 @default.
• W2155987029 hasConcept C71924100 @default.
• W2155987029 hasConcept C86803240 @default.
• W2155987029 hasConceptScore W2155987029C118487528 @default.
• W2155987029 hasConceptScore W2155987029C142724271 @default.
• W2155987029 hasConceptScore W2155987029C169760540 @default.
• W2155987029 hasConceptScore W2155987029C186198217 @default.
• W2155987029 hasConceptScore W2155987029C2776925932 @default.
• W2155987029 hasConceptScore W2155987029C2777093970 @default.
• W2155987029 hasConceptScore W2155987029C2778230176 @default.
• W2155987029 hasConceptScore W2155987029C2778650512 @default.
• W2155987029 hasConceptScore W2155987029C2778818243 @default.
• W2155987029 hasConceptScore W2155987029C2779134260 @default.
• W2155987029 hasConceptScore W2155987029C2780827179 @default.
• W2155987029 hasConceptScore W2155987029C2781040256 @default.
• W2155987029 hasConceptScore W2155987029C71924100 @default.
• W2155987029 hasConceptScore W2155987029C86803240 @default.
• W2155987029 hasIssue "1" @default.
• W2155987029 hasLocation W21559870291 @default.
• W2155987029 hasLocation W21559870292 @default.
• W2155987029 hasLocation W21559870293 @default.
• W2155987029 hasLocation W21559870294 @default.
• W2155987029 hasLocation W21559870295 @default.

• next