Thomas Franz Erich Willnow

GAS1 is required for NOTCH-dependent facilitation of SHH signaling in the ventral forebrain neuroepithelium

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Standard

GAS1 is required for NOTCH-dependent facilitation of SHH signaling in the ventral forebrain neuroepithelium. / Marczenke, Maike; Sunaga-Franze, Daniele Yumi; Popp, Oliver et al.

In: Development (Cambridge), Vol. 148, No. 21, 11.2021.

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaperJournal articleResearchpeer-review

Harvard

Marczenke, M, Sunaga-Franze, DY, Popp, O, Althaus, IW, Sauer, S, Mertins, P, Christ, A, Allen, BL & Willnow, TE 2021, 'GAS1 is required for NOTCH-dependent facilitation of SHH signaling in the ventral forebrain neuroepithelium', Development (Cambridge), vol. 148, no. 21. https://doi.org/10.1242/dev.200080

APA

Marczenke, M., Sunaga-Franze, D. Y., Popp, O., Althaus, I. W., Sauer, S., Mertins, P., Christ, A., Allen, B. L., & Willnow, T. E. (2021). GAS1 is required for NOTCH-dependent facilitation of SHH signaling in the ventral forebrain neuroepithelium. Development (Cambridge), 148(21). https://doi.org/10.1242/dev.200080

CBE

Marczenke M, Sunaga-Franze DY, Popp O, Althaus IW, Sauer S, Mertins P, Christ A, Allen BL, Willnow TE. 2021. GAS1 is required for NOTCH-dependent facilitation of SHH signaling in the ventral forebrain neuroepithelium. Development (Cambridge). 148(21). https://doi.org/10.1242/dev.200080

MLA

Vancouver

Marczenke M, Sunaga-Franze DY, Popp O, Althaus IW, Sauer S, Mertins P et al. GAS1 is required for NOTCH-dependent facilitation of SHH signaling in the ventral forebrain neuroepithelium. Development (Cambridge). 2021 Nov;148(21). https://doi.org/10.1242/dev.200080

Author

Marczenke, Maike ; Sunaga-Franze, Daniele Yumi ; Popp, Oliver et al. / GAS1 is required for NOTCH-dependent facilitation of SHH signaling in the ventral forebrain neuroepithelium. In: Development (Cambridge). 2021 ; Vol. 148, No. 21.

Bibtex

@article{a776e562eca84b41858a8efba1b40def,
title = "GAS1 is required for NOTCH-dependent facilitation of SHH signaling in the ventral forebrain neuroepithelium",
abstract = "Growth arrest-specific 1 (GAS1) acts as a co-receptor to patched 1, promoting sonic hedgehog (SHH) signaling in the developing nervous system. GAS1 mutations in humans and animal models result in forebrain and craniofacial malformations, defects ascribed to a function for GAS1 in SHH signaling during early neurulation. Here, we confirm loss of SHH activity in the forebrain neuroepithelium in GAS1-deficient mice and in induced pluripotent stem cell-derived cell models of human neuroepithelial differentiation. However, our studies document that this defect can be attributed, at least in part, to a novel role for GAS1 in facilitating NOTCH signaling, which is essential to sustain a persistent SHH activity domain in the forebrain neuroepithelium. GAS1 directly binds NOTCH1, enhancing ligand-induced processing of the NOTCH1 intracellular domain, which drives NOTCH pathway activity in the developing forebrain. Our findings identify a unique role for GAS1 in integrating NOTCH and SHH signal reception in neuroepithelial cells, and they suggest that loss of GAS1-dependent NOTCH1 activation contributes to forebrain malformations in individuals carrying GAS1 mutations.",
keywords = "Forebrain organizer region, HH co-receptors, Holoprosencephaly, Neuroepithelial precursor cells, NOTCH intracellular domain",
author = "Maike Marczenke and Sunaga-Franze, {Daniele Yumi} and Oliver Popp and Althaus, {Irene W.} and Sascha Sauer and Philipp Mertins and Annabel Christ and Allen, {Benjamin L.} and Willnow, {Thomas E.}",
note = "Publisher Copyright: {\textcopyright} 2021. Published by The Company of Biologists Ltd",
year = "2021",
month = nov,
doi = "10.1242/dev.200080",
language = "English",
volume = "148",
journal = "Development (Cambridge, England)",
issn = "0950-1991",
publisher = "The Company of Biologists",
number = "21",

}

RIS

TY - JOUR

T1 - GAS1 is required for NOTCH-dependent facilitation of SHH signaling in the ventral forebrain neuroepithelium

AU - Marczenke, Maike

AU - Sunaga-Franze, Daniele Yumi

AU - Popp, Oliver

AU - Althaus, Irene W.

AU - Sauer, Sascha

AU - Mertins, Philipp

AU - Christ, Annabel

AU - Allen, Benjamin L.

AU - Willnow, Thomas E.

N1 - Publisher Copyright: © 2021. Published by The Company of Biologists Ltd

PY - 2021/11

Y1 - 2021/11

N2 - Growth arrest-specific 1 (GAS1) acts as a co-receptor to patched 1, promoting sonic hedgehog (SHH) signaling in the developing nervous system. GAS1 mutations in humans and animal models result in forebrain and craniofacial malformations, defects ascribed to a function for GAS1 in SHH signaling during early neurulation. Here, we confirm loss of SHH activity in the forebrain neuroepithelium in GAS1-deficient mice and in induced pluripotent stem cell-derived cell models of human neuroepithelial differentiation. However, our studies document that this defect can be attributed, at least in part, to a novel role for GAS1 in facilitating NOTCH signaling, which is essential to sustain a persistent SHH activity domain in the forebrain neuroepithelium. GAS1 directly binds NOTCH1, enhancing ligand-induced processing of the NOTCH1 intracellular domain, which drives NOTCH pathway activity in the developing forebrain. Our findings identify a unique role for GAS1 in integrating NOTCH and SHH signal reception in neuroepithelial cells, and they suggest that loss of GAS1-dependent NOTCH1 activation contributes to forebrain malformations in individuals carrying GAS1 mutations.

AB - Growth arrest-specific 1 (GAS1) acts as a co-receptor to patched 1, promoting sonic hedgehog (SHH) signaling in the developing nervous system. GAS1 mutations in humans and animal models result in forebrain and craniofacial malformations, defects ascribed to a function for GAS1 in SHH signaling during early neurulation. Here, we confirm loss of SHH activity in the forebrain neuroepithelium in GAS1-deficient mice and in induced pluripotent stem cell-derived cell models of human neuroepithelial differentiation. However, our studies document that this defect can be attributed, at least in part, to a novel role for GAS1 in facilitating NOTCH signaling, which is essential to sustain a persistent SHH activity domain in the forebrain neuroepithelium. GAS1 directly binds NOTCH1, enhancing ligand-induced processing of the NOTCH1 intracellular domain, which drives NOTCH pathway activity in the developing forebrain. Our findings identify a unique role for GAS1 in integrating NOTCH and SHH signal reception in neuroepithelial cells, and they suggest that loss of GAS1-dependent NOTCH1 activation contributes to forebrain malformations in individuals carrying GAS1 mutations.

KW - Forebrain organizer region

KW - HH co-receptors

KW - Holoprosencephaly

KW - Neuroepithelial precursor cells

KW - NOTCH intracellular domain

UR - http://www.scopus.com/inward/record.url?scp=85120789106&partnerID=8YFLogxK

U2 - 10.1242/dev.200080

DO - 10.1242/dev.200080

M3 - Journal article

C2 - 34698766

AN - SCOPUS:85120789106

VL - 148

JO - Development (Cambridge, England)

JF - Development (Cambridge, England)

SN - 0950-1991

IS - 21

ER -