Matches in SemOpenAlex for { <https://semopenalex.org/work/W2143742350> ?p ?o ?g. }
- W2143742350 endingPage "1804" @default.
- W2143742350 startingPage "1792" @default.
- W2143742350 abstract "ABSTRACT Hutchinson-Gilford progeria syndrome (HGPS) is a human progeroid disease caused by a point mutation on the LMNA gene. We reported previously that the accumulation of the nuclear envelope protein SUN1 contributes to HGPS nuclear aberrancies. However, the mechanism by which interactions between mutant lamin A (also known as progerin or LAΔ50) and SUN1 produce HGPS cellular phenotypes requires further elucidation. Using light and electron microscopy, this study demonstrated that SUN1 contributes to progerin-elicited structural changes in the nuclear envelope and the endoplasmic reticulum (ER) network. We further identified two domains through which full-length lamin A associates with SUN1, and determined that the farnesylated cysteine within the CaaX motif of lamin A has a stronger affinity for SUN1 than does the lamin A region containing amino acids 607 to 656. Farnesylation of progerin enhanced its interaction with SUN1 and reduced SUN1 mobility, thereby promoting the aberrant recruitment of progerin to the ER membrane during postmitotic assembly of the nuclear envelope, resulting in the accumulation of SUN1 over consecutive cellular divisions. These results indicate that the dysregulated interaction of SUN1 and progerin in the ER during nuclear envelope reformation determines the progression of HGPS." @default.
- W2143742350 created "2016-06-24" @default.
- W2143742350 creator A5000101243 @default.
- W2143742350 creator A5006668892 @default.
- W2143742350 creator A5017779853 @default.
- W2143742350 creator A5032968846 @default.
- W2143742350 creator A5041166904 @default.
- W2143742350 creator A5044218966 @default.
- W2143742350 creator A5056278493 @default.
- W2143742350 creator A5075283925 @default.
- W2143742350 creator A5077739639 @default.
- W2143742350 date "2014-04-15" @default.
- W2143742350 modified "2023-09-27" @default.
- W2143742350 title "Dysregulated interactions between lamin A and SUN1 induce abnormalities in the nuclear envelope and endoplasmic reticulum in progeric laminopathies" @default.
- W2143742350 cites W1964551999 @default.
- W2143742350 cites W1970338621 @default.
- W2143742350 cites W1970771850 @default.
- W2143742350 cites W1973032436 @default.
- W2143742350 cites W1978274156 @default.
- W2143742350 cites W1982281628 @default.
- W2143742350 cites W1986669334 @default.
- W2143742350 cites W1995390978 @default.
- W2143742350 cites W2001971523 @default.
- W2143742350 cites W2008282952 @default.
- W2143742350 cites W2009306385 @default.
- W2143742350 cites W2010491688 @default.
- W2143742350 cites W2012502527 @default.
- W2143742350 cites W2013167944 @default.
- W2143742350 cites W2016268789 @default.
- W2143742350 cites W2019575698 @default.
- W2143742350 cites W2027798436 @default.
- W2143742350 cites W2030276925 @default.
- W2143742350 cites W2038011290 @default.
- W2143742350 cites W2038711165 @default.
- W2143742350 cites W2038761244 @default.
- W2143742350 cites W2043485308 @default.
- W2143742350 cites W2050838528 @default.
- W2143742350 cites W2055522522 @default.
- W2143742350 cites W2057493648 @default.
- W2143742350 cites W2063456960 @default.
- W2143742350 cites W2066442023 @default.
- W2143742350 cites W2066501944 @default.
- W2143742350 cites W2072595531 @default.
- W2143742350 cites W2072810104 @default.
- W2143742350 cites W2073460054 @default.
- W2143742350 cites W2076228191 @default.
- W2143742350 cites W2081288756 @default.
- W2143742350 cites W2085821267 @default.
- W2143742350 cites W2086227125 @default.
- W2143742350 cites W2094501936 @default.
- W2143742350 cites W2098484182 @default.
- W2143742350 cites W2103286704 @default.
- W2143742350 cites W2103803573 @default.
- W2143742350 cites W2111819985 @default.
- W2143742350 cites W2116633927 @default.
- W2143742350 cites W2119526996 @default.
- W2143742350 cites W2122393694 @default.
- W2143742350 cites W2125307667 @default.
- W2143742350 cites W2127708563 @default.
- W2143742350 cites W2128353741 @default.
- W2143742350 cites W2129446626 @default.
- W2143742350 cites W2133469432 @default.
- W2143742350 cites W2136329963 @default.
- W2143742350 cites W2141334538 @default.
- W2143742350 cites W2142780571 @default.
- W2143742350 cites W2143185290 @default.
- W2143742350 cites W2145283260 @default.
- W2143742350 cites W2149225679 @default.
- W2143742350 cites W2156426014 @default.
- W2143742350 cites W2156812533 @default.
- W2143742350 cites W2162288425 @default.
- W2143742350 cites W2163504716 @default.
- W2143742350 cites W2167546995 @default.
- W2143742350 cites W254147912 @default.
- W2143742350 doi "https://doi.org/10.1242/jcs.139683" @default.
- W2143742350 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/24522183" @default.
- W2143742350 hasPublicationYear "2014" @default.
- W2143742350 type Work @default.
- W2143742350 sameAs 2143742350 @default.
- W2143742350 citedByCount "60" @default.
- W2143742350 countsByYear W21437423502014 @default.
- W2143742350 countsByYear W21437423502015 @default.
- W2143742350 countsByYear W21437423502016 @default.
- W2143742350 countsByYear W21437423502017 @default.
- W2143742350 countsByYear W21437423502018 @default.
- W2143742350 countsByYear W21437423502019 @default.
- W2143742350 countsByYear W21437423502020 @default.
- W2143742350 countsByYear W21437423502021 @default.
- W2143742350 countsByYear W21437423502022 @default.
- W2143742350 countsByYear W21437423502023 @default.
- W2143742350 crossrefType "journal-article" @default.
- W2143742350 hasAuthorship W2143742350A5000101243 @default.
- W2143742350 hasAuthorship W2143742350A5006668892 @default.
- W2143742350 hasAuthorship W2143742350A5017779853 @default.
- W2143742350 hasAuthorship W2143742350A5032968846 @default.
- W2143742350 hasAuthorship W2143742350A5041166904 @default.
- W2143742350 hasAuthorship W2143742350A5044218966 @default.
- W2143742350 hasAuthorship W2143742350A5056278493 @default.