000009227 001__ 9227 000009227 005__ 20240328125236.0 000009227 0247_ $$2DOI$$a10.6083/bk128b60s 000009227 037__ $$aIR 000009227 041__ $$aeng 000009227 245__ $$aCharacterizing DRG axon phenotypes in a novel Lfng mutant 000009227 260__ $$bOregon Health and Science University 000009227 269__ $$a2021 000009227 336__ $$aAbstract 000009227 520__ $$aDuring embryonic development, structures such as the vertebral column, ribs, trunk muscles, and meninges arise from blocks of mesoderm called somites that surround the neural tube, a precursor to the central nervous system. Precise regulation of the Notch signaling pathway is required for establishing somite boundaries and patterning. Mutations in Lunatic Fringe (Lfng), a fucose-specific beta 1,3-N acetyltransferase that regulates Notch signaling, results in abnormal spacing of the somites and their derivatives, resulting in stereotypical skeletal malformations and spondylocostal dysostosis, a recessive disorder that results in malformations of the vertebral column and ribs resulting in shortened trunks and respiratory difficulties. In the present study, we sought to understand how DRG axon branching is affected throughout development and whether specific subtypes of DRG neurons are affected in LfngC20R mutants. We also examined whether the axon guidance phenotype is DRG-autonomous or due to alterations in the surrounding environment. 000009227 540__ $$fCC BY 000009227 542__ $$fIn copyright - joint owners 000009227 650__ $$aGlycosyltransferases$$029330 000009227 650__ $$aSomites$$031398 000009227 650__ $$aEmbryonic Development$$035917 000009227 650__ $$aSpinal Cord$$026279 000009227 650__ $$aMesoderm$$022089 000009227 650__ $$aReceptors, Notch$$037046 000009227 6531_ $$aembryo 000009227 6531_ $$alunatic fringe 000009227 692__ $$aVollum Institute$$041509 000009227 7001_ $$aLuong, Nina$$uOregon Health and Science University$$041354 000009227 7001_ $$aPomaville, Matthew$$uOregon Health and Science University$$041354 000009227 7001_ $$aWright, Kevin$$uOregon Health and Science University$$041354 000009227 711__ $$aResearch Week$$uOregon Health and Science University$$d2021 000009227 8564_ $$9ebebf7e6-5a32-466f-b5e7-3b77117370ff$$s80620$$uhttps://digitalcollections.ohsu.edu/record/9227/files/Luong-Nina-OHSU-ResearchWeek-2021.pdf 000009227 905__ $$a/rest/prod/bk/12/8b/60/bk128b60s 000009227 980__ $$aResearch Week