MassIVE MSV000081568

Partial Public PXD007869

Analysis of peripheral neuron protein expression in the absence of Ikbkap/Elp1

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Description

Familial dysautonomia (FD) results from mutation in IKBKAP/ELP1, a gene encoding the scaffolding protein for the Elongator complex. This highly conserved complex is required for the methoxy-carbonyl-methyl (mcm5) modification of uridines located in the wobble position of tRNA molecules (U34). In FD, the peripheral nervous system is particularly devastated by loss of IKBKAP. Here we investigate protein expression within the dorsal root ganglia (DRG) of a mouse model of FD. In this model, Ikbkap is selectively deleted in the neural crest lineage using a Wnt1-Cre transgene and floxed Ikbkap. DRG were collected and pooled from seven Ikbkap conditional knockout (Wnt1-Cre;IkbkapLoxP/LoxP) and seven control E17.5 mice and analyzed via UHPLC-MS/MS. [dataset license: CC0 1.0 Universal (CC0 1.0)]

Keywords: Mouse, dorsal root ganglia, DRG, Ikbkap, IKAP, Elongator, familial dysautonomia, FD

Contact

Principal Investigators:
(in alphabetical order) 		Lynn D George, Montana State University Billings, United States
Submitting User: jgoffena

Publications

Goffena J, Lefcort F, Zhang Y, Lehrmann E, Chaverra M, Felig J, Walters J, Buksch R, Becker KG, George L.
Elongator and codon bias regulate protein levels in mammalian peripheral neurons.
Nat Commun. 2018 Mar 1;9(1):889. Epub 2018 Mar 1.

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