FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2139797302> ?p ?o ?g. }

• W2139797302 endingPage "18116" @default.
• W2139797302 startingPage "18111" @default.
• W2139797302 abstract "Zmpste24 is a metalloproteinase required for the processing of prelamin A to lamin A, a structural component of the nuclear lamina. Zmpste24 deficiency results in the accumulation of prelamin A within cells, a complete loss of mature lamin A, and misshapen nuclear envelopes. Zmpste24 -deficient ( Zmpste24 –/– ) mice exhibit retarded growth, alopecia, micrognathia, dental abnormalities, osteolytic lesions in bones, and osteoporosis, which are phenotypes shared with Hutchinson–Gilford progeria syndrome, a human disease caused by the synthesis of a mutant prelamin A that cannot undergo processing to lamin A. Zmpste24 –/– mice also develop muscle weakness. We hypothesized that prelamin A might be toxic and that its accumulation in Zmpste24 –/– mice is responsible for all of the disease phenotypes. We further hypothesized that Zmpste24 –/– mice with half-normal levels of prelamin A ( Zmpste24 –/– mice with one Lmna knockout allele) would be subjected to less toxicity and be protected from disease. Thus, we bred and analyzed Zmpste24 –/– Lmna +/– mice. As expected, prelamin A levels in Zmpste24 –/– Lmna +/– cells were significantly reduced. Zmpste24 –/– Lmna +/– mice were entirely normal, lacking all disease phenotypes, and misshapen nuclei were less frequent in Zmpste24 –/– Lmna +/– cells than in Zmpste24 –/– cells. These data suggest that prelamin A is toxic and that reducing its levels by as little as 50% provides striking protection from disease." @default.
• W2139797302 created "2016-06-24" @default.
• W2139797302 creator A5011939199 @default.
• W2139797302 creator A5019944871 @default.
• W2139797302 creator A5038969060 @default.
• W2139797302 creator A5043462798 @default.
• W2139797302 creator A5056038080 @default.
• W2139797302 creator A5056994112 @default.
• W2139797302 creator A5065757281 @default.
• W2139797302 creator A5068606823 @default.
• W2139797302 creator A5069893656 @default.
• W2139797302 creator A5085755071 @default.
• W2139797302 creator A5087964537 @default.
• W2139797302 creator A5091681921 @default.
• W2139797302 date "2004-12-17" @default.
• W2139797302 modified "2023-10-06" @default.
• W2139797302 title "Heterozygosity for <i>Lmna</i> deficiency eliminates the progeria-like phenotypes in <i>Zmpste24</i> -deficient mice" @default.
• W2139797302 cites W1001811995 @default.
• W2139797302 cites W1487741670 @default.
• W2139797302 cites W1708471163 @default.
• W2139797302 cites W1964551999 @default.
• W2139797302 cites W1988227606 @default.
• W2139797302 cites W2006846732 @default.
• W2139797302 cites W2007472609 @default.
• W2139797302 cites W2036973053 @default.
• W2139797302 cites W2037999013 @default.
• W2139797302 cites W2042168939 @default.
• W2139797302 cites W2061331409 @default.
• W2139797302 cites W2071425478 @default.
• W2139797302 cites W2074997168 @default.
• W2139797302 cites W2079643856 @default.
• W2139797302 cites W2127708563 @default.
• W2139797302 cites W2136490523 @default.
• W2139797302 cites W2136979747 @default.
• W2139797302 cites W2137564725 @default.
• W2139797302 cites W2143277187 @default.
• W2139797302 cites W2143963878 @default.
• W2139797302 cites W2153340375 @default.
• W2139797302 cites W2169413646 @default.
• W2139797302 cites W2172254982 @default.
• W2139797302 cites W4247425028 @default.
• W2139797302 doi "https://doi.org/10.1073/pnas.0408558102" @default.
• W2139797302 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/536056" @default.
• W2139797302 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/15608054" @default.
• W2139797302 hasPublicationYear "2004" @default.
• W2139797302 type Work @default.
• W2139797302 sameAs 2139797302 @default.
• W2139797302 citedByCount "189" @default.
• W2139797302 countsByYear W21397973022012 @default.
• W2139797302 countsByYear W21397973022013 @default.
• W2139797302 countsByYear W21397973022014 @default.
• W2139797302 countsByYear W21397973022015 @default.
• W2139797302 countsByYear W21397973022016 @default.
• W2139797302 countsByYear W21397973022017 @default.
• W2139797302 countsByYear W21397973022018 @default.
• W2139797302 countsByYear W21397973022019 @default.
• W2139797302 countsByYear W21397973022020 @default.
• W2139797302 countsByYear W21397973022021 @default.
• W2139797302 countsByYear W21397973022022 @default.
• W2139797302 countsByYear W21397973022023 @default.
• W2139797302 crossrefType "journal-article" @default.
• W2139797302 hasAuthorship W2139797302A5011939199 @default.
• W2139797302 hasAuthorship W2139797302A5019944871 @default.
• W2139797302 hasAuthorship W2139797302A5038969060 @default.
• W2139797302 hasAuthorship W2139797302A5043462798 @default.
• W2139797302 hasAuthorship W2139797302A5056038080 @default.
• W2139797302 hasAuthorship W2139797302A5056994112 @default.
• W2139797302 hasAuthorship W2139797302A5065757281 @default.
• W2139797302 hasAuthorship W2139797302A5068606823 @default.
• W2139797302 hasAuthorship W2139797302A5069893656 @default.
• W2139797302 hasAuthorship W2139797302A5085755071 @default.
• W2139797302 hasAuthorship W2139797302A5087964537 @default.
• W2139797302 hasAuthorship W2139797302A5091681921 @default.
• W2139797302 hasBestOaLocation W21397973022 @default.
• W2139797302 hasConcept C104317684 @default.
• W2139797302 hasConcept C126322002 @default.
• W2139797302 hasConcept C127716648 @default.
• W2139797302 hasConcept C134018914 @default.
• W2139797302 hasConcept C179093185 @default.
• W2139797302 hasConcept C2778465436 @default.
• W2139797302 hasConcept C2780654407 @default.
• W2139797302 hasConcept C29512474 @default.
• W2139797302 hasConcept C502942594 @default.
• W2139797302 hasConcept C54355233 @default.
• W2139797302 hasConcept C71924100 @default.
• W2139797302 hasConcept C86339819 @default.
• W2139797302 hasConcept C86803240 @default.
• W2139797302 hasConcept C94912109 @default.
• W2139797302 hasConceptScore W2139797302C104317684 @default.
• W2139797302 hasConceptScore W2139797302C126322002 @default.
• W2139797302 hasConceptScore W2139797302C127716648 @default.
• W2139797302 hasConceptScore W2139797302C134018914 @default.
• W2139797302 hasConceptScore W2139797302C179093185 @default.
• W2139797302 hasConceptScore W2139797302C2778465436 @default.
• W2139797302 hasConceptScore W2139797302C2780654407 @default.
• W2139797302 hasConceptScore W2139797302C29512474 @default.
• W2139797302 hasConceptScore W2139797302C502942594 @default.
• W2139797302 hasConceptScore W2139797302C54355233 @default.

• next