FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3204940322> ?p ?o ?g. }

• W3204940322 abstract "ABSTRACT The common null polymorphism (R577X) in the ACTN3 gene is present in over 1.5 billion people worldwide and results in the absence of the protein α-actinin-3 from the Z-discs of fast-twitch skeletal muscle fibres. We have previously reported that this polymorphism is a modifier of dystrophin deficient Duchenne Muscular Dystrophy. To investigate the mechanism underlying this we use a double knockout (dk) Actn3KO / mdx (dKO) mouse model which lacks both dystrophin and sarcomere α-actinin-3. We used dKO mice and mdx dystrophic mice at 12 months (aged) to investigate the correlation between morphological changes to the fast-twitch dKO EDL and the reduction in force deficit produced by an in vitro eccentric contraction protocol. In the aged dKO mouse we found a marked reduction in fibre branching complexity that correlated with protection from eccentric contraction induced force deficit. Complex branches in the aged dKO EDL fibres (28%) were substantially reduced compared to aged mdx EDL fibres (68%) and this correlates with a graded force loss over three eccentric contractions for dKO muscles (∼35% after first contraction, ∼66% overall) compared to an abrupt drop in mdx upon the first eccentric contraction (∼73% after first contraction, ∼89% after three contractions). In dKO protection from eccentric contraction damage was linked with a doubling of SERCA1 pump density the EDL. We propose that the increased oxidative metabolism of fast-twitch glycolytic fibres characteristic of the null polymorphism (R577X) and increase in SR Ca 2+ pump proteins reduces muscle fibre branching and decreases susceptibility to eccentric injury in the dystrophinopathies." @default.
• W3204940322 created "2021-10-11" @default.
• W3204940322 creator A5015504090 @default.
• W3204940322 creator A5029141514 @default.
• W3204940322 creator A5062507539 @default.
• W3204940322 creator A5080324325 @default.
• W3204940322 date "2021-10-01" @default.
• W3204940322 modified "2023-10-16" @default.
• W3204940322 title "Loss of α-actinin-3 confers protection from eccentric contraction damage in fast-twitch EDL muscles from aged mdx dystrophic mice by reducing pathological fibre branching" @default.
• W3204940322 cites W1501820843 @default.
• W3204940322 cites W1594995535 @default.
• W3204940322 cites W1957055985 @default.
• W3204940322 cites W1964690048 @default.
• W3204940322 cites W1971292110 @default.
• W3204940322 cites W1978282477 @default.
• W3204940322 cites W1982930017 @default.
• W3204940322 cites W1987711577 @default.
• W3204940322 cites W1989566133 @default.
• W3204940322 cites W1998398326 @default.
• W3204940322 cites W2002561225 @default.
• W3204940322 cites W2008310176 @default.
• W3204940322 cites W2010152384 @default.
• W3204940322 cites W2031451946 @default.
• W3204940322 cites W2032275523 @default.
• W3204940322 cites W2033359534 @default.
• W3204940322 cites W2036567058 @default.
• W3204940322 cites W2037638256 @default.
• W3204940322 cites W2040099865 @default.
• W3204940322 cites W2050376269 @default.
• W3204940322 cites W2055374521 @default.
• W3204940322 cites W2077209938 @default.
• W3204940322 cites W2079864904 @default.
• W3204940322 cites W2086368761 @default.
• W3204940322 cites W2094601234 @default.
• W3204940322 cites W2094835866 @default.
• W3204940322 cites W2095681865 @default.
• W3204940322 cites W2101579071 @default.
• W3204940322 cites W2103409944 @default.
• W3204940322 cites W2105484902 @default.
• W3204940322 cites W2111945300 @default.
• W3204940322 cites W2112604043 @default.
• W3204940322 cites W2114603492 @default.
• W3204940322 cites W2118149354 @default.
• W3204940322 cites W2130025116 @default.
• W3204940322 cites W2132155798 @default.
• W3204940322 cites W2133501942 @default.
• W3204940322 cites W2135675109 @default.
• W3204940322 cites W2136909562 @default.
• W3204940322 cites W2137714739 @default.
• W3204940322 cites W2137848437 @default.
• W3204940322 cites W2150553525 @default.
• W3204940322 cites W2157112460 @default.
• W3204940322 cites W2158694936 @default.
• W3204940322 cites W2162938177 @default.
• W3204940322 cites W2165382119 @default.
• W3204940322 cites W2165593789 @default.
• W3204940322 cites W2171492836 @default.
• W3204940322 cites W2206766868 @default.
• W3204940322 cites W2220646252 @default.
• W3204940322 cites W2256959533 @default.
• W3204940322 cites W2274001545 @default.
• W3204940322 cites W2292568679 @default.
• W3204940322 cites W2344980798 @default.
• W3204940322 cites W2584800155 @default.
• W3204940322 cites W2787839776 @default.
• W3204940322 cites W2802957102 @default.
• W3204940322 cites W2894967856 @default.
• W3204940322 cites W3007768457 @default.
• W3204940322 cites W3088559812 @default.
• W3204940322 cites W3093170144 @default.
• W3204940322 cites W3106290375 @default.
• W3204940322 cites W3116239948 @default.
• W3204940322 cites W3132160447 @default.
• W3204940322 cites W3142808969 @default.
• W3204940322 cites W3152397041 @default.
• W3204940322 cites W3195703638 @default.
• W3204940322 cites W3197816368 @default.
• W3204940322 cites W97046176 @default.
• W3204940322 doi "https://doi.org/10.1101/2021.10.01.462704" @default.
• W3204940322 hasPublicationYear "2021" @default.
• W3204940322 type Work @default.
• W3204940322 sameAs 3204940322 @default.
• W3204940322 citedByCount "0" @default.
• W3204940322 crossrefType "posted-content" @default.
• W3204940322 hasAuthorship W3204940322A5015504090 @default.
• W3204940322 hasAuthorship W3204940322A5029141514 @default.
• W3204940322 hasAuthorship W3204940322A5062507539 @default.
• W3204940322 hasAuthorship W3204940322A5080324325 @default.
• W3204940322 hasBestOaLocation W32049403221 @default.
• W3204940322 hasConcept C100784341 @default.
• W3204940322 hasConcept C105702510 @default.
• W3204940322 hasConcept C121332964 @default.
• W3204940322 hasConcept C12554922 @default.
• W3204940322 hasConcept C126322002 @default.
• W3204940322 hasConcept C133702464 @default.
• W3204940322 hasConcept C134018914 @default.
• W3204940322 hasConcept C142669718 @default.
• W3204940322 hasConcept C1491633281 @default.
• W3204940322 hasConcept C163415756 @default.
• W3204940322 hasConcept C185592680 @default.

• next