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Metadata

Name
The ESCRT-III protein CHMP1A mediates secretion of sonic hedgehog on a novel class of extracellular vesicles - GPL16791
Repository
Gemma
Identifier
gemma.experiment:19746
Description
Extracellular vesicles (EVs) enable cell-to-cell communication in the nervous system essential for development and adult function. Endosomal Sorting Complex Required for Transport (ESCRT) complex proteins regulate EV formation and release. Recent work shows loss of function (LOF) mutations in, CHMP1A, which encodes one ESCRT-III member, cause autosomal recessive microcephaly with pontocerebellar hypoplasia in humans (Mochida et al., 2012). Here we show CHMP1A is required for maintenance of progenitors in human cerebral organoids and that mouse Chmp1a is required for progenitor proliferation in cortex and cerebellum and specifically for sonic hedgehog (SHH) mediated proliferation through SHH secretion. CHMP1A mutation reduces intraluminal vesicle (ILV) formation in multivesicular bodies (MVBs), and EV release. SHH protein is present on a subset of EVs marked by a unique set of proteins we call ART-EVs. CHMP1A’s requirement in formation of ART-EVs and other EVs provides a model to elucidate EV functions in multiple brain processes.
At time of import, last updated (by provider) on:

Contributors: ; [Michael E Coulter, Harald Hess, Richard Smith, Maria K Lehtinen, Christopher A Walsh, Sarah Cianferani, François Delalande, Elaine T Lim, Frank M Jacobs, Cristina Dorobantu, Eric T Wong, Vijay Ganesh, Hart G Lidov, Thorsten Schlaeger, Tomas Kirchhausen, Wei-Chung Lee, Gerrald A Lodewijk, Monica L Calicchio, Edward Yang, Raphael Gaudin, Ganesh Mochida, C S Xu, David Haussler, Song Pang, Dilenny M Gonzalez]
Data or Study Types
gene expression
Keywords
functional genomics
Source Organization
University of British Columbia
Access Conditions
available
Access Hyperlink
www.chibi.ubc.ca/Gemma/expressionExperiment/showExpressionExperiment.html?id=19746

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