Matches in SemOpenAlex for { <https://semopenalex.org/work/W4214755161> ?p ?o ?g. }
- W4214755161 endingPage "282" @default.
- W4214755161 startingPage "270" @default.
- W4214755161 abstract "Menkes disease (MNK) is a rare X-linked recessive disease, caused by mutations in the copper transporting ATP7A gene that is required for copper homeostasis. MNK patients experience various clinical symptoms including neurological defects that are closely related to the prognosis of MNK patients. Neural stem cells (NSCs) in the hippocampal dentate gyrus (DG) produce new neurons throughout life, and defects in DG neurogenesis are often correlated with cognitive and behavioral problems. However, neurodevelopmental defects in the DG during postnatal period in MNK have not been understood yet.Mottled-brindled (MoBr/y) mice (MNK mice) and littermate controls were used in this study. In vivo microCT imaging and immunohistochemistry results demonstrate that blood vasculatures in hippocampus are abnormally decreased in MNK mice. Furthermore, postnatal establishment of NSC population and their neurogenesis are severely compromised in the DG of MNK mice. In addition, in vitro analyses using hippocampal neurosphere culture followed by immunocytochemistry and immunoblotting suggest that neurogenesis from MNK NSCs is also significantly compromised, corresponding to defective neurogenic gene expression in MNK derived neurons.Our study is the first reports demonstrating that improper expansion of the postnatal NSC population followed by significant reduction of neurogenesis may contribute to neurodevelopmental symptoms in MNK. In conclusion, our results provide new insight into early neurodevelopmental defects in MNK and emphasize the needs for early diagnosis and new therapeutic strategies in the postnatal central nerve system damage of MNK patients." @default.
- W4214755161 created "2022-03-02" @default.
- W4214755161 creator A5000614597 @default.
- W4214755161 creator A5022145302 @default.
- W4214755161 creator A5036605496 @default.
- W4214755161 creator A5044044590 @default.
- W4214755161 creator A5046979869 @default.
- W4214755161 creator A5066332230 @default.
- W4214755161 creator A5069866514 @default.
- W4214755161 creator A5078775403 @default.
- W4214755161 creator A5091554389 @default.
- W4214755161 date "2022-02-28" @default.
- W4214755161 modified "2023-10-14" @default.
- W4214755161 title "Abnormal Development of Neural Stem Cell Niche in the Dentate Gyrus of Menkes Disease" @default.
- W4214755161 cites W1442455712 @default.
- W4214755161 cites W1529036811 @default.
- W4214755161 cites W1533389761 @default.
- W4214755161 cites W1972893985 @default.
- W4214755161 cites W1973373552 @default.
- W4214755161 cites W1993651887 @default.
- W4214755161 cites W2003503502 @default.
- W4214755161 cites W2006057621 @default.
- W4214755161 cites W2019937832 @default.
- W4214755161 cites W2024174118 @default.
- W4214755161 cites W2026393786 @default.
- W4214755161 cites W2036729969 @default.
- W4214755161 cites W2042169215 @default.
- W4214755161 cites W2046683077 @default.
- W4214755161 cites W2051021067 @default.
- W4214755161 cites W2051187289 @default.
- W4214755161 cites W2066547209 @default.
- W4214755161 cites W2072383565 @default.
- W4214755161 cites W2079797122 @default.
- W4214755161 cites W2085536739 @default.
- W4214755161 cites W2085870232 @default.
- W4214755161 cites W2089847501 @default.
- W4214755161 cites W2091391415 @default.
- W4214755161 cites W2092341724 @default.
- W4214755161 cites W2098999053 @default.
- W4214755161 cites W2103497218 @default.
- W4214755161 cites W2107069175 @default.
- W4214755161 cites W2115891976 @default.
- W4214755161 cites W2128211642 @default.
- W4214755161 cites W2135046848 @default.
- W4214755161 cites W2139208934 @default.
- W4214755161 cites W2151298438 @default.
- W4214755161 cites W2165867905 @default.
- W4214755161 cites W2167036056 @default.
- W4214755161 cites W2170057620 @default.
- W4214755161 cites W2268784712 @default.
- W4214755161 cites W2324497588 @default.
- W4214755161 cites W2402416000 @default.
- W4214755161 cites W2462934906 @default.
- W4214755161 cites W29858201 @default.
- W4214755161 doi "https://doi.org/10.15283/ijsc21088" @default.
- W4214755161 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/35220279" @default.
- W4214755161 hasPublicationYear "2022" @default.
- W4214755161 type Work @default.
- W4214755161 citedByCount "0" @default.
- W4214755161 crossrefType "journal-article" @default.
- W4214755161 hasAuthorship W4214755161A5000614597 @default.
- W4214755161 hasAuthorship W4214755161A5022145302 @default.
- W4214755161 hasAuthorship W4214755161A5036605496 @default.
- W4214755161 hasAuthorship W4214755161A5044044590 @default.
- W4214755161 hasAuthorship W4214755161A5046979869 @default.
- W4214755161 hasAuthorship W4214755161A5066332230 @default.
- W4214755161 hasAuthorship W4214755161A5069866514 @default.
- W4214755161 hasAuthorship W4214755161A5078775403 @default.
- W4214755161 hasAuthorship W4214755161A5091554389 @default.
- W4214755161 hasBestOaLocation W42147551611 @default.
- W4214755161 hasConcept C136834591 @default.
- W4214755161 hasConcept C142724271 @default.
- W4214755161 hasConcept C148762608 @default.
- W4214755161 hasConcept C169760540 @default.
- W4214755161 hasConcept C178790620 @default.
- W4214755161 hasConcept C185592680 @default.
- W4214755161 hasConcept C2776464000 @default.
- W4214755161 hasConcept C2777581567 @default.
- W4214755161 hasConcept C2781161787 @default.
- W4214755161 hasConcept C28328180 @default.
- W4214755161 hasConcept C2908647359 @default.
- W4214755161 hasConcept C2991729501 @default.
- W4214755161 hasConcept C4746552 @default.
- W4214755161 hasConcept C544778455 @default.
- W4214755161 hasConcept C71924100 @default.
- W4214755161 hasConcept C86803240 @default.
- W4214755161 hasConcept C95444343 @default.
- W4214755161 hasConcept C99454951 @default.
- W4214755161 hasConceptScore W4214755161C136834591 @default.
- W4214755161 hasConceptScore W4214755161C142724271 @default.
- W4214755161 hasConceptScore W4214755161C148762608 @default.
- W4214755161 hasConceptScore W4214755161C169760540 @default.
- W4214755161 hasConceptScore W4214755161C178790620 @default.
- W4214755161 hasConceptScore W4214755161C185592680 @default.
- W4214755161 hasConceptScore W4214755161C2776464000 @default.
- W4214755161 hasConceptScore W4214755161C2777581567 @default.
- W4214755161 hasConceptScore W4214755161C2781161787 @default.
- W4214755161 hasConceptScore W4214755161C28328180 @default.