<<< Full experiment listing

PXD004923

PXD004923 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleProteomic and genomic characterization of a yeast model for Ogden Syndrome
DescriptionNaa10 is a N-terminal acetyltransferase that, in a complex with its auxiliary subunit Naa15, co-translationally acetylates the -amino group of newly synthetized proteins as they emerge from the ribosome. Roughly 40-50% of the human proteome is acetylated by Naa10, rendering this an enzyme with one of the most broad substrate ranges known. Recently, we reported an X-linked disorder of infancy, Ogden syndrome, in two families harboring a c.109T>C (p.Ser37Pro) variant in NAA10. In the present study we performed in-depth characterization of a yeast model of Ogden syndrome. Stress tests and proteomic analyses suggest that the S37P mutation disrupts Naa10 function and reduces cellular fitness during heat shock, possibly due to dysregulation of chaperone expression and accumulation. Microarray and RNA-seq revealed a pseudo-diploid gene expression profile in Naa10 cells, likely responsible for a mating defect. In conclusion, the data presented here further support the disruptive nature of the S37P/Ogden mutation and identify affected cellular processes potentially contributing to the severe phenotype seen in Ogden syndrome.
HostingRepositoryPRIDE
AnnounceDate2016-09-28
AnnouncementXMLSubmission_2016-09-28_07:43:19.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterKeith Rivera
SpeciesList scientific name: Saccharomyces cerevisiae (Baker's yeast); NCBI TaxID: 4932;
ModificationListmethylthiolated residue; iTRAQ8plex-116 reporter+balance reagent acylated residue; monohydroxylated residue; deamidated residue
InstrumentLTQ Orbitrap Velos
Dataset History
RevisionDatetimeStatusChangeLog Entry
02016-09-12 01:57:10ID requested
12016-09-28 07:43:20announced
Publication List
D, ö, rfel MJ, Fang H, Crain J, Klingener M, Weiser J, Lyon GJ, Proteomic and genomic characterization of a yeast model for Ogden syndrome. Yeast, 34(1):19-37(2017) [pubmed]
Keyword List
curator keyword: Biological
submitter keyword: Ogden Syndrome
Contact List
Gholson J. Lyon
contact affiliationStanley Institute for Cognitive Genomics, One Bungtown Road, Cold Spring Harbor Laboratory, Cold Spring Harbor, NY, USA
contact emailGholsonJLyon@gmail.com
lab head
Keith Rivera
contact affiliationCold Spring Harbor Laboratory
contact emailkrivera@cshl.edu
dataset submitter
Full Dataset Link List
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/2016/09/PXD004923
PRIDE project URI
Repository Record List
[ + ]