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PXD049178

PXD049178 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleDysregulated Extracellular Matrix and Increased Stiffness in Lissencephaly Brain Organoid Models
DescriptionLIS1 mutations are known to cause lissencephaly, a neurological disorder characterized by the absence of cerebral cortical convolutions. Here we show that brain organoids with LIS1 mutations exhibit increased expression of extracellular matrix (ECM) proteins, which are less organized. The mechanical properties of the mutant organoids demonstrated increased stiffness and steady-state stiffness. These changes are associated with dysregulated mRNA and microRNA. Short-term treatment of the mutant organoids, but not the control ones, with the catalytic subunit of MMP2, which proteolytically cleaves ECM, reduced stiffness. The changes were associated with changes in water diffusion measured by MRI (Magnetic Resonance Imaging) and gene expression. We generated a model incorporating the differences in the mutant's and control brain organoid composition and organization that matches and explains our findings. Our study provides insights into the importance of the composition and organization of the ECM during human brain development and the role of LIS1 in brain structure.
HostingRepositoryPRIDE
AnnounceDate2025-03-11
AnnouncementXMLSubmission_2025-03-11_01:30:16.501.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterYishai Levin
SpeciesList scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationListmonohydroxylated residue; iodoacetamide derivatized residue
InstrumenttimsTOF Pro
Dataset History
RevisionDatetimeStatusChangeLog Entry
02024-02-05 04:27:54ID requested
12025-03-11 01:30:16announced
Publication List
Dataset with its publication pending
Keyword List
submitter keyword: Extracellular Matrix, Brain, organoid
Contact List
Orly Reiner
contact affiliationDepartment of Molecular Genetics Weizmann Institute of Science
contact emailorly.reiner@weizmann.ac.il
lab head
Yishai Levin
contact affiliationWeizmann Institute of Science, Israel
contact emailyishai.levin@gmail.com
dataset submitter
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Dataset FTP location
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