sonic hedgehog signaling update

[Guest guest]

(for our new members and others not aware of the research on sonic

hedgehog protein and signaling and the implication on nerves and

genetics, there is a simply written page on this in our files

/files/Research%20projects/

this was written for CMT Today magazine and appeared about this time.

Members of have been following Hh since abbout 2001. ~ Gretchen)

Research from Development. 2004 May;131(9):2149-59.

FKBP8 is a negative regulator of mouse sonic hedgehog signaling in

neural tissues.

Bulgakov OV, Eggenschwiler JT, Hong DH, KV, Li T.

The Berman-Gund Laboratory for the Study of Retinal Degenerations,

Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, MA

02114, USA.

Sonic hedgehog (SHH) is a secreted morphogen that regulates the

patterning and growth of many tissues in the developing mouse embryo,

including the central nervous system (CNS). We show that a member of the

FK506-binding protein family, FKBP8, is an essential antagonist of SHH

signaling in CNS development. Loss of FKBP8 causes ectopic and

ligand-independent activation of the Shh pathway, leading to expansion

of ventral cell fates in the posterior neural tube and suppression of

eye development. Although it is expressed broadly, FKBP8 is required to

antagonize SHH signaling primarily in neural tissues, suggesting that

hedgehog signal transduction is subject to cell-type specific modulation

during mammalian development.