FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3007238052> ?p ?o ?g. }

• W3007238052 endingPage "146" @default.
• W3007238052 startingPage "133" @default.
• W3007238052 abstract "The importance of the glomerular basement membrane (GBM) in glomerular filtration is underscored by the manifestations of Alport and Pierson syndromes, caused by defects in type IV collagen α3α4α5 and the laminin β2 chain, respectively. Lamb2 null mice, which model the most severe form of Pierson syndrome, exhibit proteinuria prior to podocyte foot process effacement and are therefore useful for studying GBM permselectivity. We hypothesize that some LAMB2 missense mutations that cause mild forms of Pierson syndrome induce GBM destabilization with delayed effects on podocytes. While generating a CRISPR/Cas9-mediated analogue of a human LAMB2 missense mutation in mice, we identified a 44-amino acid deletion (LAMB2-Del44) within the laminin N-terminal domain, a domain mediating laminin polymerization. Laminin heterotrimers containing LAMB2-Del44 exhibited a 90% reduction in polymerization in vitro that was partially rescued by type IV collagen and nidogen. Del44 mice showed albuminuria at 1.8-6.0 g/g creatinine (ACR) at one to two months, plateauing at an average 200 g/g ACR at 3.7 months, when GBM thickening and hallmarks of nephrotic syndrome were first observed. Despite the massive albuminuria, some Del44 mice survived for up to 15 months. Blood urea nitrogen was modestly elevated at seven-nine months. Eight to nine-month-old Del44 mice exhibited glomerulosclerosis and interstitial fibrosis. Similar to Lamb2-/- mice, proteinuria preceded foot process effacement. Foot processes were widened but not effaced at one-two months despite the high ACRs. At three months some individual foot processes were still observed amid widespread effacement. Thus, our chronic model of nephrotic syndrome may prove useful to study filtration mechanisms, long-term proteinuria with preserved kidney function, and to test therapeutics." @default.
• W3007238052 created "2020-03-06" @default.
• W3007238052 creator A5004907055 @default.
• W3007238052 creator A5007016006 @default.
• W3007238052 creator A5019591664 @default.
• W3007238052 creator A5026117179 @default.
• W3007238052 creator A5048492882 @default.
• W3007238052 creator A5072027400 @default.
• W3007238052 creator A5076535178 @default.
• W3007238052 date "2020-07-01" @default.
• W3007238052 modified "2023-09-26" @default.
• W3007238052 title "A deletion in the N-terminal polymerizing domain of laminin β2 is a new mouse model of chronic nephrotic syndrome" @default.
• W3007238052 cites W1496520030 @default.
• W3007238052 cites W1616451600 @default.
• W3007238052 cites W166223212 @default.
• W3007238052 cites W1696043613 @default.
• W3007238052 cites W183565456 @default.
• W3007238052 cites W1966435431 @default.
• W3007238052 cites W1967605282 @default.
• W3007238052 cites W1971370141 @default.
• W3007238052 cites W1976696904 @default.
• W3007238052 cites W1978643970 @default.
• W3007238052 cites W1982387497 @default.
• W3007238052 cites W1996775227 @default.
• W3007238052 cites W2006140887 @default.
• W3007238052 cites W2014510950 @default.
• W3007238052 cites W2018215777 @default.
• W3007238052 cites W2023961186 @default.
• W3007238052 cites W2033233404 @default.
• W3007238052 cites W2038514844 @default.
• W3007238052 cites W2046071266 @default.
• W3007238052 cites W2048502130 @default.
• W3007238052 cites W2057915502 @default.
• W3007238052 cites W2057958444 @default.
• W3007238052 cites W2058691614 @default.
• W3007238052 cites W2059709989 @default.
• W3007238052 cites W2065235940 @default.
• W3007238052 cites W2071108036 @default.
• W3007238052 cites W2076166690 @default.
• W3007238052 cites W2083620770 @default.
• W3007238052 cites W2099489196 @default.
• W3007238052 cites W2108546852 @default.
• W3007238052 cites W2109354358 @default.
• W3007238052 cites W2122792426 @default.
• W3007238052 cites W2124565234 @default.
• W3007238052 cites W2129649143 @default.
• W3007238052 cites W2138256890 @default.
• W3007238052 cites W2143355463 @default.
• W3007238052 cites W2143987989 @default.
• W3007238052 cites W2147143604 @default.
• W3007238052 cites W2148171171 @default.
• W3007238052 cites W2154558576 @default.
• W3007238052 cites W2159862904 @default.
• W3007238052 cites W2164934883 @default.
• W3007238052 cites W2212959237 @default.
• W3007238052 cites W2256212941 @default.
• W3007238052 cites W2300796136 @default.
• W3007238052 cites W2341561883 @default.
• W3007238052 cites W2346347415 @default.
• W3007238052 cites W2407386961 @default.
• W3007238052 cites W2409881191 @default.
• W3007238052 cites W2561907496 @default.
• W3007238052 cites W2588366281 @default.
• W3007238052 cites W2772986061 @default.
• W3007238052 cites W2781260670 @default.
• W3007238052 cites W2793237837 @default.
• W3007238052 cites W2801914936 @default.
• W3007238052 cites W2883300568 @default.
• W3007238052 cites W4241456778 @default.
• W3007238052 doi "https://doi.org/10.1016/j.kint.2020.01.033" @default.
• W3007238052 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/7311232" @default.
• W3007238052 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/32456966" @default.
• W3007238052 hasPublicationYear "2020" @default.
• W3007238052 type Work @default.
• W3007238052 sameAs 3007238052 @default.
• W3007238052 citedByCount "9" @default.
• W3007238052 countsByYear W30072380522020 @default.
• W3007238052 countsByYear W30072380522021 @default.
• W3007238052 countsByYear W30072380522022 @default.
• W3007238052 crossrefType "journal-article" @default.
• W3007238052 hasAuthorship W3007238052A5004907055 @default.
• W3007238052 hasAuthorship W3007238052A5007016006 @default.
• W3007238052 hasAuthorship W3007238052A5019591664 @default.
• W3007238052 hasAuthorship W3007238052A5026117179 @default.
• W3007238052 hasAuthorship W3007238052A5048492882 @default.
• W3007238052 hasAuthorship W3007238052A5072027400 @default.
• W3007238052 hasAuthorship W3007238052A5076535178 @default.
• W3007238052 hasBestOaLocation W30072380522 @default.
• W3007238052 hasConcept C104317684 @default.
• W3007238052 hasConcept C126322002 @default.
• W3007238052 hasConcept C134018914 @default.
• W3007238052 hasConcept C142724271 @default.
• W3007238052 hasConcept C159641895 @default.
• W3007238052 hasConcept C189165786 @default.
• W3007238052 hasConcept C2776115139 @default.
• W3007238052 hasConcept C2776174234 @default.
• W3007238052 hasConcept C2776908417 @default.
• W3007238052 hasConcept C2777390665 @default.

• next