Neuroscience

Too Soon

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Science  29 Nov 2013:
Vol. 342, Issue 6162, pp. 1020
DOI: 10.1126/science.342.6162.1020-b
CREDIT: L. GEORGE ET AL., PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES 110, 46 (30 OCTOBER 2013)

Familial dysautonomia arises from the inappropriate splicing of an mRNA that encodes a kinase inhibitor; this affects sensory and autonomic functions of the peripheral nervous system, and those affected die young. George et al. have looked at the function of the inhibitor in the neural crest lineage, which is the source of much of the peripheral nervous system. In mice without the inhibitor, there were fewer than normal neurons in the superior cervical ganglia and dorsal root ganglia (DRG). Particularly depleted were neurons expressing the neurotrophin receptor TrkA. Their analysis of embryonic mouse development showed that the inhibitor was present in progenitor cells and in postmitotic neurons representing the second wave of neurogenesis in the DRG, the wave that produces TrkA-positive nociceptors and thermoreceptors. Without the inhibitor, this subgroup of progenitors differentiates too early, resulting in a precocious excess of these sensory neurons but depleting progenitors, so that the numbers of neurons fail to keep pace with subsequent development.

Proc. Natl. Acad. Sci. U.S.A. 110, 10.1073/pnas.1308596110 (2013).

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