Ectopic sonic hedgehog signaling impairs telencephalic dorsal midline development: implication for human holoprosencephaly.

Ectopic sonic hedgehog signaling impairs telencephalic dorsal midline development: implication for human holoprosencephaly. Research Abstract Details 

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Ectopic sonic hedgehog signaling impairs telencephalic dorsal midline development: implication for human holoprosencephaly. Abstract Text:

Xi Huang,Ying Litingtung,Chin Chiang,

Holoprosencephaly (HPE) is the most common developmental anomaly of the human forebrain, and in its severe form, the cerebral hemispheres fail to completely separate into two distinct halves. Although disruption of ventral forebrain induction is thought to underlie most HPE cases, a subset of HPE patients exhibits preferential dysgenesis of forebrain dorsal midline structures with unknown etiology. In this study, we show that Sonic hedgehog (Shh) lacking cholesterol moiety in one allele (ShhN/+) in mice can elicit ectopic Shh signaling in early telencephalon to induce ventral progenitor marker expression in the cortical region and impair telencephalic dorsal midline development. Prolonged ectopic ShhN signaling impaired Bmp and Wnt signaling from the dorsal patterning center through upregulation of Fgf8, leading to augmented cell proliferation, decreased cell death and impaired roof plate morphogenesis. Accordingly, ShhN/+ mutant telencephalic dorsal midline structures, including cortical hem, hippocampus and choroid plexus, either failed to form or were hypoplastic. Strikingly, ShhN/+ mutants displayed a spectrum of phenotypic features such as failure of anterior cerebral hemisphere to divide, hydrocephalus and cleft palate which have been observed in a human patient with milder HPE predicted to produce SHHN protein due to a truncation mutation in one SHH allele. We propose that elevated ectopic Shh signaling can impair dorsal telencephalic midline morphogenesis, and lead to non-cleavage of midline structures mimicking human HPE with dorsal midline defects.

Ectopic sonic hedgehog signaling impairs telencephalic dorsal midline development: implication for human holoprosencephaly. Publishing Authors By Initials

X Huang,Y Litingtung,C Chiang,

For similar peptides: intercellular signaling peptides and proteins: wnt proteins research abstracts see: peptides: intercellular signaling peptides and proteins: wnt proteins research

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Ectopic sonic hedgehog signaling impairs telencephalic dorsal midline development: implication for human holoprosencephaly. Journal Published:

PUBLICATION TYPE: Research Support, N.I.H., Extr

Journal: Human molecular genetics

VOLUME: 16

Page Numbers: 1454-68

Journal Abbreviation: Hum. Mol. Genet.

ISSN: 0964-6906

DAY: 27

MONTH: 04

YEAR: 2007

Ectopic sonic hedgehog signaling impairs telencephalic dorsal midline development: implication for human holoprosencephaly. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 9208958

Ectopic sonic hedgehog signaling impairs telencephalic dorsal midline development: implication for human holoprosencephaly. Keywords Mesh Terms:

KEYWORDS: Wnt Proteins

MESH TERMS: metabolism

Chemical & Substance for Abstract: Ectopic sonic hedgehog signaling impairs telencephalic dorsal midline development: implication for human holoprosencephaly. Information

Substance Name: Fibroblast Growth Factor 8

Registry Number: 148997-75-5

Grant and Affiliation Information for Ectopic sonic hedgehog signaling impairs telencephalic dorsal midline development: implication for human holoprosencephaly.

AFFILIATION: Department of Cell and Developmental Biology, Vanderbilt University Medical Center, 4114 MRB III, Nashville, TN 37232, USA.

Country: England

AGENCY: United States NINDS

GRANT: NS42205

ACRONYM: NS

MEDLINETA: Hum Mol Genet

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