PXD011961

PXD011961 is an original dataset announced via ProteomeXchange.

Title	Shifts in Ribosome Engagement Impact Key Gene Sets in Neurodevelopment and Rett Syndrome Neurons
Description	Translation of many transcripts is highly regulated in the developing brain, and disturbance of translational regulation machinery contributes to neurodevelopmental disorders. In neural progenitor cells, for example, several critical pro-differentiation genes are transcribed, but their translation is repressed to allow rapid translation when appropriate signals to differentiate are received. This layer of translational regulation makes it challenging to directly correlate RNA and protein levels in stem cells and neurons. During early neural development, translation is regulated by several pathways that can impact neuron fate and function. The mTOR-mediated signaling pathway plays a crucial role in the induction of neuron differentiation, axon and dendrite development, and gliogenesis, while being key in the maintenance of pluripotent and neural stem cells {Wang:2013hg}{Agrawal:2014ek}{Ka:2014fq}. Dysregulation of the translation repressor eIF4E-binding protein 2 (4EBP2), a downstream target of mTORC1, leads to an increased ratio of excitatory to inhibitory synaptic inputs and autistic-like behaviors {Gkogkas:2013fh}. The Fragile X Mental Retardation Protein (FMRP), which is encoded by the FMR1 gene {Verkerk:1991hu}, is an RNA binding protein (RBP) that regulates translation through multiple mechanisms {Richter:2015ii}. Loss of expression of FMR1 causes Fragile X Syndrome (FXS), the most common inherited intellectual disability as well as the most prevalent single-gene cause of autism spectrum disorder (ASD). FMRP typically functions as a translational repressor {Li:2001ds} and some studies suggest that FXS results from an inability of neurons to achieve regulated local translation, particularly in response to stimuli {Richter:2015ii}. This suggests important roles for control of translation in stem cells and neurons, and an association with significant risk for neurodevelopmental disorders.
HostingRepository	PRIDE
AnnounceDate	2020-05-07
AnnouncementXML	Submission_2020-05-07_02:23:51.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Ugljesa Djuric
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	No PTMs are included in the dataset
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2018-12-06 05:57:29	ID requested
⏵ 1	2020-05-07 02:33:16	announced

Rodrigues DC, Mufteev M, Weatheritt RJ, Djuric U, Ha KCH, Ross PJ, Wei W, Piekna A, Sartori MA, Byres L, Mok RSF, Zaslavsky K, Pasceri P, Diamandis P, Morris Q, Blencowe BJ, Ellis J, Shifts in Ribosome Engagement Impact Key Gene Sets in Neurodevelopment and Ubiquitination in Rett Syndrome. Cell Rep, 30(12):4179-4196.e11(2020) [pubmed]

curator keyword: Biological, Biomedical

submitter keyword: Rett syndrome, RNAseq, MS proteomics

Phedias Diamandis
contact affiliation	UHN
contact email	p.diamandis@mail.utoronto.ca
lab head
Ugljesa Djuric
contact affiliation	Mt. Sinai Hospital
contact email	ugljesa.djuric@mail.utoronto.ca
dataset submitter

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.pride.ebi.ac.uk/pride/data/archive/2020/05/PXD011961

PRIDE project URI

• PRIDE
  1. PXD011961
     1. Label: PRIDE project
     2. Name: Shifts in Ribosome Engagement Impact Key Gene Sets in Neurodevelopment and Rett Syndrome Neurons