FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2785386055> ?p ?o ?g. }

• W2785386055 endingPage "65" @default.
• W2785386055 startingPage "55" @default.
• W2785386055 abstract "Mutations to the BSCL2 gene disrupt the protein seipin and cause the most severe form of congenital generalised lipodystrophy (CGL). Affected individuals exhibit a near complete loss of white adipose tissue (WAT) and suffer from metabolic disease. Seipin is critical for adipocyte development in culture and mice with germline disruption to Bscl2 recapitulate the effects of BSCL2 disruption in humans. Here we examined whether loss of Bscl2 specifically in developing adipocytes in vivo is sufficient to prevent adipose tissue development and cause all features observed with congenital BSCL2 disruption. We generated and characterised a novel mouse model of Bscl2 deficiency in developing adipocytes (Ad-B2(−/−)) using the adipose-specific Adiponectin-Cre line. We demonstrate that Ad-B2(−/−) mice display early onset lipodystrophy, in common with congenital Bscl2 null mice and CGL2 patients. However, glucose intolerance, insulin resistance, and severe hepatic steatosis are not apparent. Food intake and energy expenditure are unchanged, but Ad-B2(−/−) mice exhibit significantly altered substrate utilisation. We also find differential effects of seipin loss between specific adipose depots revealing new insights regarding their varied characteristics. When fed a high-fat diet, Ad-B2(−/−) mice entirely fail to expand adipose mass but remain glucose tolerant. Our findings demonstrate that disruption of Bscl2 specifically in developing adipocytes is sufficient to cause the early-onset generalised lipodystrophy observed in patients with mutations in BSCL2. However, this significant reduction in adipose mass does not cause the overt metabolic dysfunction seen in Bscl2 knockout mice, even following a high-fat diet challenge." @default.
• W2785386055 created "2018-02-23" @default.
• W2785386055 creator A5014023198 @default.
• W2785386055 creator A5017237941 @default.
• W2785386055 creator A5019178220 @default.
• W2785386055 creator A5026623515 @default.
• W2785386055 creator A5030409595 @default.
• W2785386055 creator A5048328515 @default.
• W2785386055 creator A5049569282 @default.
• W2785386055 creator A5058412586 @default.
• W2785386055 creator A5066862424 @default.
• W2785386055 creator A5083857860 @default.
• W2785386055 creator A5085181522 @default.
• W2785386055 creator A5091519450 @default.
• W2785386055 date "2018-04-01" @default.
• W2785386055 modified "2023-10-02" @default.
• W2785386055 title "Adipose specific disruption of seipin causes early-onset generalised lipodystrophy and altered fuel utilisation without severe metabolic disease" @default.
• W2785386055 cites W1847008334 @default.
• W2785386055 cites W1963384348 @default.
• W2785386055 cites W1964743358 @default.
• W2785386055 cites W1964873147 @default.
• W2785386055 cites W1965126423 @default.
• W2785386055 cites W1980143302 @default.
• W2785386055 cites W1985649195 @default.
• W2785386055 cites W2004861205 @default.
• W2785386055 cites W2006877714 @default.
• W2785386055 cites W2009172922 @default.
• W2785386055 cites W2009330135 @default.
• W2785386055 cites W2013317654 @default.
• W2785386055 cites W2014932253 @default.
• W2785386055 cites W2027258013 @default.
• W2785386055 cites W2027753197 @default.
• W2785386055 cites W2035335697 @default.
• W2785386055 cites W2035559736 @default.
• W2785386055 cites W2036463408 @default.
• W2785386055 cites W2073830546 @default.
• W2785386055 cites W2074633912 @default.
• W2785386055 cites W2085355922 @default.
• W2785386055 cites W2085544097 @default.
• W2785386055 cites W2099745966 @default.
• W2785386055 cites W2099983274 @default.
• W2785386055 cites W2100012264 @default.
• W2785386055 cites W2103370215 @default.
• W2785386055 cites W2118648486 @default.
• W2785386055 cites W2120117726 @default.
• W2785386055 cites W2126327417 @default.
• W2785386055 cites W2134014535 @default.
• W2785386055 cites W2136238917 @default.
• W2785386055 cites W2138956168 @default.
• W2785386055 cites W2139104247 @default.
• W2785386055 cites W2140689550 @default.
• W2785386055 cites W2146193579 @default.
• W2785386055 cites W2155268144 @default.
• W2785386055 cites W2158225793 @default.
• W2785386055 cites W2172297599 @default.
• W2785386055 cites W2263803825 @default.
• W2785386055 cites W2346774190 @default.
• W2785386055 cites W2351429842 @default.
• W2785386055 cites W2396528434 @default.
• W2785386055 cites W2532979816 @default.
• W2785386055 cites W2547006549 @default.
• W2785386055 cites W2550704454 @default.
• W2785386055 cites W2568435816 @default.
• W2785386055 cites W2765091292 @default.
• W2785386055 doi "https://doi.org/10.1016/j.molmet.2018.01.019" @default.
• W2785386055 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/5985228" @default.
• W2785386055 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/29459250" @default.
• W2785386055 hasPublicationYear "2018" @default.
• W2785386055 type Work @default.
• W2785386055 sameAs 2785386055 @default.
• W2785386055 citedByCount "33" @default.
• W2785386055 countsByYear W27853860552018 @default.
• W2785386055 countsByYear W27853860552019 @default.
• W2785386055 countsByYear W27853860552020 @default.
• W2785386055 countsByYear W27853860552021 @default.
• W2785386055 countsByYear W27853860552022 @default.
• W2785386055 countsByYear W27853860552023 @default.
• W2785386055 crossrefType "journal-article" @default.
• W2785386055 hasAuthorship W2785386055A5014023198 @default.
• W2785386055 hasAuthorship W2785386055A5017237941 @default.
• W2785386055 hasAuthorship W2785386055A5019178220 @default.
• W2785386055 hasAuthorship W2785386055A5026623515 @default.
• W2785386055 hasAuthorship W2785386055A5030409595 @default.
• W2785386055 hasAuthorship W2785386055A5048328515 @default.
• W2785386055 hasAuthorship W2785386055A5049569282 @default.
• W2785386055 hasAuthorship W2785386055A5058412586 @default.
• W2785386055 hasAuthorship W2785386055A5066862424 @default.
• W2785386055 hasAuthorship W2785386055A5083857860 @default.
• W2785386055 hasAuthorship W2785386055A5085181522 @default.
• W2785386055 hasAuthorship W2785386055A5091519450 @default.
• W2785386055 hasBestOaLocation W27853860551 @default.
• W2785386055 hasConcept C126322002 @default.
• W2785386055 hasConcept C134018914 @default.
• W2785386055 hasConcept C142462285 @default.
• W2785386055 hasConcept C151955695 @default.
• W2785386055 hasConcept C171089720 @default.
• W2785386055 hasConcept C203014093 @default.
• W2785386055 hasConcept C2776175234 @default.

• next