FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2951453058> ?p ?o ?g. }

• W2951453058 endingPage "3561" @default.
• W2951453058 startingPage "3545" @default.
• W2951453058 abstract "Combined germline and somatic second-hit inactivating mutations of the RASA1 gene, which encodes a negative regulator of the Ras signaling pathway, cause blood and lymphatic vascular lesions in the human autosomal-dominant vascular disorder capillary malformation–arteriovenous malformation (CM-AVM). How RASA1 mutations in endothelial cells (ECs) result in vascular lesions in CM-AVM is unknown. Here, using different murine models of RASA1 deficiency, we found that RASA1 was essential for the survival of ECs during developmental angiogenesis, in which primitive vascular plexuses are remodeled into hierarchical vascular networks. RASA1 was required for EC survival during developmental angiogenesis, because it was necessary for export of collagen IV from ECs and deposition in vascular basement membranes. In the absence of RASA1, dysregulated Ras/MAPK signal transduction in ECs resulted in impaired folding of collagen IV and its retention in the endoplasmic reticulum (ER), leading to EC death. Remarkably, the chemical chaperone 4-phenylbutyric acid and small-molecule inhibitors of MAPK and 2-oxoglutarate–dependent collagen IV–modifying enzymes rescued ER retention of collagen IV and EC apoptosis and resulted in normal developmental angiogenesis. These findings have important implications for a better understanding of the molecular pathogenesis of CM-AVM and possible means of treatment." @default.
• W2951453058 created "2019-06-27" @default.
• W2951453058 creator A5006212222 @default.
• W2951453058 creator A5012402551 @default.
• W2951453058 creator A5031574868 @default.
• W2951453058 creator A5062964311 @default.
• W2951453058 creator A5087696769 @default.
• W2951453058 date "2019-08-05" @default.
• W2951453058 modified "2023-10-09" @default.
• W2951453058 title "RASA1-dependent cellular export of collagen IV controls blood and lymphatic vascular development" @default.
• W2951453058 cites W125553237 @default.
• W2951453058 cites W1596750122 @default.
• W2951453058 cites W1862556865 @default.
• W2951453058 cites W1884981594 @default.
• W2951453058 cites W1973096186 @default.
• W2951453058 cites W1976161806 @default.
• W2951453058 cites W1978369267 @default.
• W2951453058 cites W1982650516 @default.
• W2951453058 cites W1984577233 @default.
• W2951453058 cites W1994540908 @default.
• W2951453058 cites W1996405087 @default.
• W2951453058 cites W1998828538 @default.
• W2951453058 cites W2001231863 @default.
• W2951453058 cites W2001879388 @default.
• W2951453058 cites W2001999652 @default.
• W2951453058 cites W2006771504 @default.
• W2951453058 cites W2006931104 @default.
• W2951453058 cites W2007066284 @default.
• W2951453058 cites W2017352211 @default.
• W2951453058 cites W2023962283 @default.
• W2951453058 cites W2026075246 @default.
• W2951453058 cites W2029425171 @default.
• W2951453058 cites W2029924206 @default.
• W2951453058 cites W2032756767 @default.
• W2951453058 cites W2040670989 @default.
• W2951453058 cites W2041039856 @default.
• W2951453058 cites W2043074419 @default.
• W2951453058 cites W2043508733 @default.
• W2951453058 cites W2043623672 @default.
• W2951453058 cites W2049902469 @default.
• W2951453058 cites W2068707753 @default.
• W2951453058 cites W2068896961 @default.
• W2951453058 cites W2071959332 @default.
• W2951453058 cites W2075787053 @default.
• W2951453058 cites W2079576402 @default.
• W2951453058 cites W2082855409 @default.
• W2951453058 cites W2087719510 @default.
• W2951453058 cites W2105182945 @default.
• W2951453058 cites W2112078762 @default.
• W2951453058 cites W2126756606 @default.
• W2951453058 cites W2140215777 @default.
• W2951453058 cites W2141636978 @default.
• W2951453058 cites W2149117196 @default.
• W2951453058 cites W2150629192 @default.
• W2951453058 cites W2155495006 @default.
• W2951453058 cites W2155501369 @default.
• W2951453058 cites W2157910877 @default.
• W2951453058 cites W2161162374 @default.
• W2951453058 cites W2164903526 @default.
• W2951453058 cites W2169164452 @default.
• W2951453058 cites W2169675124 @default.
• W2951453058 cites W2192080449 @default.
• W2951453058 cites W2293557323 @default.
• W2951453058 cites W2476542314 @default.
• W2951453058 cites W2569842642 @default.
• W2951453058 cites W2616736391 @default.
• W2951453058 cites W2627083739 @default.
• W2951453058 cites W2762413087 @default.
• W2951453058 cites W2764223953 @default.
• W2951453058 cites W2781825595 @default.
• W2951453058 cites W2890006342 @default.
• W2951453058 doi "https://doi.org/10.1172/jci124917" @default.
• W2951453058 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6715364" @default.
• W2951453058 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/31185000" @default.
• W2951453058 hasPublicationYear "2019" @default.
• W2951453058 type Work @default.
• W2951453058 sameAs 2951453058 @default.
• W2951453058 citedByCount "38" @default.
• W2951453058 countsByYear W29514530582019 @default.
• W2951453058 countsByYear W29514530582020 @default.
• W2951453058 countsByYear W29514530582021 @default.
• W2951453058 countsByYear W29514530582022 @default.
• W2951453058 countsByYear W29514530582023 @default.
• W2951453058 crossrefType "journal-article" @default.
• W2951453058 hasAuthorship W2951453058A5006212222 @default.
• W2951453058 hasAuthorship W2951453058A5012402551 @default.
• W2951453058 hasAuthorship W2951453058A5031574868 @default.
• W2951453058 hasAuthorship W2951453058A5062964311 @default.
• W2951453058 hasAuthorship W2951453058A5087696769 @default.
• W2951453058 hasBestOaLocation W29514530581 @default.
• W2951453058 hasConcept C104317684 @default.
• W2951453058 hasConcept C134018914 @default.
• W2951453058 hasConcept C143065580 @default.
• W2951453058 hasConcept C158617107 @default.
• W2951453058 hasConcept C181152851 @default.
• W2951453058 hasConcept C185592680 @default.
• W2951453058 hasConcept C203014093 @default.
• W2951453058 hasConcept C2776193487 @default.

• next