PXD008833-1

PXD008833 is an original dataset announced via ProteomeXchange.

Title	Zc3h10 is a novel mitochondrial regulator
Description	Mitochondria are the energy-generating hubs of the cell. In spite of considerable advances, our understanding of the factors that regulate the molecular circuits that govern mitochondrial function remains incomplete. Using a genome-wide functional screen, we have identified the poorly characterized protein Zinc finger CCCH-type containing 10 (Zc3h10) as regulator of mitochondrial physiology. We show that Zc3h10 is upregulated during physiological mitochondriogenesis such as myoblasts differentiation into myotubes. Zc3h10 overexpression boosts mitochondrial function and promotes myoblasts differentiation. On the other hand, depletion of Zc3h10 results in impaired myoblasts differentiation, mitochondrial dysfunction, reduced expression of electron transport chain (ETC) subunits and blunted TCA cycle flux. Notably, we have identified a loss-of-function mutation of Zc3h10 in humans (Tyr105 to Cys105) that is associated with increased body mass index, fat mass, fasting glucose and triglycerides. Isolated peripheral blood mononuclear cells from Cys105 homozygotes display reduced oxygen consumption rate, some ETC subunit expression and decreased levels of some TCA cycle metabolites that derive in mitochondrial dysfunction. Finally, our study identifies Zc3h10 as a novel mitochondrial regulator.
HostingRepository	PRIDE
AnnounceDate	2018-03-06
AnnouncementXML	Submission_2018-03-06_06:01:24.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	alessandro cuomo
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	No PTMs are included in the dataset
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2018-02-01 09:08:28	ID requested
⏵ 1	2018-03-06 06:01:24	announced
2	2018-03-12 02:29:41	announced	Updated publication reference for PubMed record(s): 29507079.

Dataset with its publication pending

curator keyword: Biological

submitter keyword: Zc3h10, mitochondria, energy metabolism, oxidative phosphorylation.

Nico Mitro
contact affiliation	DiSFeB, Dipartimento di Scienze Farmacologiche e Biomolecolari, Università degli Studi di Milano, Milano, 20133, Italy,
contact email	nico.mitro@unimi.it
lab head
alessandro cuomo
contact affiliation	IEO
contact email	alessandro.cuomo@ieo.it
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/2018/03/PXD008833

PRIDE project URI

• PRIDE
  1. PXD008833
     1. Label: PRIDE project
     2. Name: Zc3h10 is a novel mitochondrial regulator