PXD021385

PXD021385 is an original dataset announced via ProteomeXchange.

Title	Complexome profiling of mitochondrial protein complexes to study the molecular consequences of DNAJC30 mutations
Description	We identified the chaperone DNAJC30 as an important factor to maintain NADH:ubiquinone oxidoreductase (complex I) activity. In this complexome analysis we detected DNAJC30 in substoichiometric amounts attached to respiratory supercomplexes (I/III2/IVn and demonstrate an accumulation of complex I containing respiratory supercomplexes in the patient cell line with DNAJC30 mutations compared to the control cell line.
HostingRepository	PRIDE
AnnounceDate	2021-09-09
AnnouncementXML	Submission_2021-09-09_09:53:21.008.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Ilka Wittig
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	iodoacetamide derivatized residue
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2020-09-09 22:48:24	ID requested
⏵ 1	2021-09-09 09:53:21	announced

Stenton SL, Sheremet NL, Catarino CB, Andreeva NA, Assouline Z, Barboni P, Barel O, Berutti R, Bychkov I, Caporali L, Capristo M, Carbonelli M, Cascavilla ML, Charbel Issa P, Freisinger P, Gerber S, Ghezzi D, Graf E, Heidler J, Hempel M, Heon E, Itkis YS, Javasky E, Kaplan J, Kopajtich R, Kornblum C, Kovacs-Nagy R, Krylova TD, Kunz WS, La Morgia C, Lamperti C, Ludwig C, Malacarne PF, Maresca A, Mayr JA, Meisterknecht J, Nevinitsyna TA, Palombo F, Pode-Shakked B, Shmelkova MS, Strom TM, Tagliavini F, Tzadok M, van der Ven AT, Vignal-Clermont C, Wagner M, Zakharova EY, Zhorzholadze NV, Rozet JM, Carelli V, Tsygankova PG, Klopstock T, Wittig I, Prokisch H, Impaired complex I repair causes recessive Leber's hereditary optic neuropathy. J Clin Invest, 131(6):(2021) [pubmed]

submitter keyword: LC-MSMS, complexome profiling, oxidative phosphorylation system, complex I maintenance, complex I assembly

Holger Prokisch
contact affiliation	Research Group of Mitochondrial Disorders, Institute of Human Genetics, Technische Universität München, München, Germany
contact email	prokisch@helmholtz-muenchen.de
lab head
Ilka Wittig
contact affiliation	Functional Proteomics, Goethe University, Frankfurt am Main , Germany
contact email	wittig@med.uni-frankfurt.de
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/2021/09/PXD021385

PRIDE project URI

• PRIDE
  1. PXD021385
     1. Label: PRIDE project
     2. Name: Complexome profiling of mitochondrial protein complexes to study the molecular consequences of DNAJC30 mutations