PXD004923 is an
original dataset announced via ProteomeXchange.
Dataset Summary
Title | Proteomic and genomic characterization of a yeast model for Ogden Syndrome |
Description | Naa10 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. |
HostingRepository | PRIDE |
AnnounceDate | 2016-09-28 |
AnnouncementXML | Submission_2016-09-28_07:43:19.xml |
DigitalObjectIdentifier | |
ReviewLevel | Peer-reviewed dataset |
DatasetOrigin | Original dataset |
RepositorySupport | Unsupported dataset by repository |
PrimarySubmitter | Keith Rivera |
SpeciesList | scientific name: Saccharomyces cerevisiae (Baker's yeast); NCBI TaxID: 4932; |
ModificationList | methylthiolated residue; iTRAQ8plex-116 reporter+balance reagent acylated residue; monohydroxylated residue; deamidated residue |
Instrument | LTQ Orbitrap Velos |
Dataset History
Revision | Datetime | Status | ChangeLog Entry |
0 | 2016-09-12 01:57:10 | ID requested | |
⏵ 1 | 2016-09-28 07:43:20 | announced | |
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 affiliation | Stanley Institute for Cognitive Genomics, One Bungtown Road, Cold Spring Harbor Laboratory, Cold Spring Harbor, NY, USA |
contact email | GholsonJLyon@gmail.com |
lab head | |
Keith Rivera |
contact affiliation | Cold Spring Harbor Laboratory |
contact email | krivera@cshl.edu |
dataset submitter | |
Full Dataset Link List
Dataset FTP location
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PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD004923
- Label: PRIDE project
- Name: Proteomic and genomic characterization of a yeast model for Ogden Syndrome