Proteomics

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

ABSTRACT: 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.

OTHER RELATED OMICS DATASETS IN: GSE80165PRJNA318069PRJNA318068

INSTRUMENT(S): Q Exactive

ORGANISM(S): Mus Musculus (ncbitaxon:10090)

SUBMITTER: Lynn D George  

PROVIDER: MSV000081568 | MassIVE |

SECONDARY ACCESSION(S): PXD007869

REPOSITORIES: MassIVE

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Elongator and codon bias regulate protein levels in mammalian peripheral neurons.

Goffena Joy J   Lefcort Frances F   Zhang Yongqing Y   Lehrmann Elin E   Chaverra Marta M   Felig Jehremy J   Walters Joseph J   Buksch Richard R   Becker Kevin G KG   George Lynn L  

Nature communications 20180301 1

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 translation of codon-biased genes in lower organisms. Here we investigate whether Elongator serves a similar function in mammalian peripheral neurons, the population devastated in FD. Using codon-biased eGFP sensors, and multiplexing of codon usage with transcriptome and proteome analyses of over 6,000 genes, we iden  ...[more]

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