FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W4283375998> ?p ?o ?g. }

• W4283375998 endingPage "180" @default.
• W4283375998 startingPage "169" @default.
• W4283375998 abstract "Loss of function of the neurofibromatosis type 2 (NF2) tumor suppressor gene leads to the formation of schwannomas, meningiomas, and ependymomas, comprising ∼50% of all sporadic cases of primary nervous system tumors. NF2 syndrome is an autosomal dominant condition, with bi-allelic inactivation of germline and somatic alleles resulting in loss of function of the encoded protein merlin and activation of mammalian target of rapamycin (mTOR) pathway signaling in NF2-deficient cells. Here we describe a gene replacement approach through direct intratumoral injection of an adeno-associated virus vector expressing merlin in a novel human schwannoma model in nude mice. In culture, the introduction of an AAV1 vector encoding merlin into CRISPR-modified human NF2-null arachnoidal cells (ACs) or Schwann cells (SCs) was associated with decreased size and mTORC1 pathway activation consistent with restored merlin activity. In vivo, a single injection of AAV1-merlin directly into human NF2-null SC-derived tumors growing in the sciatic nerve of nude mice led to regression of tumors over a 10-week period, associated with a decrease in dividing cells and an increase in apoptosis, in comparison with vehicle. These studies establish that merlin re-expression via gene replacement in NF2-null schwannomas is sufficient to cause tumor regression, thereby potentially providing an effective treatment for NF2." @default.
• W4283375998 created "2022-06-25" @default.
• W4283375998 creator A5000816967 @default.
• W4283375998 creator A5002305003 @default.
• W4283375998 creator A5005191447 @default.
• W4283375998 creator A5011818435 @default.
• W4283375998 creator A5016545930 @default.
• W4283375998 creator A5016860642 @default.
• W4283375998 creator A5026837154 @default.
• W4283375998 creator A5032940778 @default.
• W4283375998 creator A5043341881 @default.
• W4283375998 creator A5045740999 @default.
• W4283375998 creator A5050547837 @default.
• W4283375998 creator A5053754777 @default.
• W4283375998 creator A5059573074 @default.
• W4283375998 creator A5059797681 @default.
• W4283375998 creator A5064371575 @default.
• W4283375998 creator A5091816977 @default.
• W4283375998 date "2022-09-01" @default.
• W4283375998 modified "2023-10-17" @default.
• W4283375998 title "Gene replacement therapy in a schwannoma mouse model of neurofibromatosis type 2" @default.
• W4283375998 cites W171436837 @default.
• W4283375998 cites W1875005970 @default.
• W4283375998 cites W1963587413 @default.
• W4283375998 cites W1967535091 @default.
• W4283375998 cites W1969345121 @default.
• W4283375998 cites W1969884064 @default.
• W4283375998 cites W1980364128 @default.
• W4283375998 cites W1991675243 @default.
• W4283375998 cites W2000522885 @default.
• W4283375998 cites W2006920577 @default.
• W4283375998 cites W2010453590 @default.
• W4283375998 cites W2012819315 @default.
• W4283375998 cites W2026304709 @default.
• W4283375998 cites W2027008782 @default.
• W4283375998 cites W2029550671 @default.
• W4283375998 cites W2050152356 @default.
• W4283375998 cites W2052713251 @default.
• W4283375998 cites W2060020667 @default.
• W4283375998 cites W2064339362 @default.
• W4283375998 cites W2099727495 @default.
• W4283375998 cites W2102448688 @default.
• W4283375998 cites W2103880807 @default.
• W4283375998 cites W2108745864 @default.
• W4283375998 cites W2115328665 @default.
• W4283375998 cites W2123301270 @default.
• W4283375998 cites W2136083637 @default.
• W4283375998 cites W2143901024 @default.
• W4283375998 cites W2144540418 @default.
• W4283375998 cites W2150706412 @default.
• W4283375998 cites W2156611910 @default.
• W4283375998 cites W2167279371 @default.
• W4283375998 cites W2167764524 @default.
• W4283375998 cites W2171634629 @default.
• W4283375998 cites W2174393453 @default.
• W4283375998 cites W2308374231 @default.
• W4283375998 cites W2355642969 @default.
• W4283375998 cites W2520817237 @default.
• W4283375998 cites W2765408594 @default.
• W4283375998 cites W2804238454 @default.
• W4283375998 cites W2888076703 @default.
• W4283375998 cites W2908004680 @default.
• W4283375998 cites W2908857457 @default.
• W4283375998 cites W2938689878 @default.
• W4283375998 cites W2945235200 @default.
• W4283375998 cites W3008199088 @default.
• W4283375998 cites W3009851500 @default.
• W4283375998 cites W3111331330 @default.
• W4283375998 cites W3121789320 @default.
• W4283375998 cites W3135935053 @default.
• W4283375998 cites W3185693930 @default.
• W4283375998 cites W4252286376 @default.
• W4283375998 cites W574235218 @default.
• W4283375998 doi "https://doi.org/10.1016/j.omtm.2022.06.012" @default.
• W4283375998 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/35846573" @default.
• W4283375998 hasPublicationYear "2022" @default.
• W4283375998 type Work @default.
• W4283375998 citedByCount "4" @default.
• W4283375998 countsByYear W42833759982022 @default.
• W4283375998 countsByYear W42833759982023 @default.
• W4283375998 crossrefType "journal-article" @default.
• W4283375998 hasAuthorship W4283375998A5000816967 @default.
• W4283375998 hasAuthorship W4283375998A5002305003 @default.
• W4283375998 hasAuthorship W4283375998A5005191447 @default.
• W4283375998 hasAuthorship W4283375998A5011818435 @default.
• W4283375998 hasAuthorship W4283375998A5016545930 @default.
• W4283375998 hasAuthorship W4283375998A5016860642 @default.
• W4283375998 hasAuthorship W4283375998A5026837154 @default.
• W4283375998 hasAuthorship W4283375998A5032940778 @default.
• W4283375998 hasAuthorship W4283375998A5043341881 @default.
• W4283375998 hasAuthorship W4283375998A5045740999 @default.
• W4283375998 hasAuthorship W4283375998A5050547837 @default.
• W4283375998 hasAuthorship W4283375998A5053754777 @default.
• W4283375998 hasAuthorship W4283375998A5059573074 @default.
• W4283375998 hasAuthorship W4283375998A5059797681 @default.
• W4283375998 hasAuthorship W4283375998A5064371575 @default.
• W4283375998 hasAuthorship W4283375998A5091816977 @default.
• W4283375998 hasBestOaLocation W42833759981 @default.

• next