PXD056807

PXD056807 is an original dataset announced via ProteomeXchange.

Title	EMG1 cooperates with GRHL3 in β-catenin–mediated surface ectoderm differentiation to regulate neural tube closure
Description	Grainyhead-like transcription factor 3 (GRHL3) directs surface ectoderm differentiation under the control of the canonical Wnt/β-catenin pathway. However, the molecular mechanisms that control nuclear GRHL3 expression through β-catenin are not fully understood. Here, we show that the essential for mitotic growth 1 (EMG1) protein constitutes a protein complex with GRHL3, and that EMG1 is required for correct nuclear localization of GRHL3, and for activation of the canonical Wnt signaling pathway. Conditional knockout mutation of Emg1 in the GRHL3-positive surface ectoderm causes neural tube defects at the level of the spinal cord, i.e. spina bifida. Additionally, the severity of compound mutant phenotypes of the Emg1 and Grhl3 genes indicates that they interact genetically in neurulation and palate development. These lines of evidence demonstrate that EMG1 cooperates with GRHL3 in β-catenin–mediated surface ectoderm differentiation. Since the EMG1 mutation causes Bowen-Conradi syndrome and the GRHL3 mutation causes Van der Woude syndrome 2, both of which are associated with neural tube dysplasia and cleft palate, our study will help to better understand the pathogenic mechanisms of these two human genetic diseases.
HostingRepository	jPOST
AnnounceDate	2025-07-01
AnnouncementXML	Submission_2025-07-01_00:19:21.656.xml
DigitalObjectIdentifier
ReviewLevel	Non peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Emi Mishiro-Sato
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	S-carboxamidomethyl-L-cysteine; alpha-amino acetylated residue; L-methionine sulfoxide
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2024-10-15 01:01:51	ID requested
⏵ 1	2025-07-01 00:19:22	announced

Dataset with its publication pending

submitter keyword: canonical Wnt/β-catenin, epidermal differentiation, neurulation, palatogenesis, Bowen-Conradi syndrome, Van der Woude syndrome 2

Chiharu Kimura-Yoshida, Isao Matsuo
lab head
Emi Mishiro-Sato
contact affiliation	WPI-ITbM, Nagoya University
dataset submitter

jPOST dataset URI

Dataset FTP location NOTE: Most web browsers have now discontinued native support for FTP access within the browser window. But you can usually install another FTP app (we recommend FileZilla) and configure your browser to launch the external application when you click on this FTP link. Or otherwise, launch an app that supports FTP (like FileZilla) and use this address: ftp://ftp.jpostdb.org/JPST003419/