PXD016816

PXD016816 is an original dataset announced via ProteomeXchange.

Title	Interactomes of WT and H1069Q variants of ATP7B
Description	The H1069Q substitution in the liver-specific copper transporter ATP7B represents the major cause of Wilson disease. The mutated ATP7B undergoes rapid degradation in the endoplasmic reticulum (ER) and fails to reach copper excretion compartments thus causing severe copper toxicosis in patients. Modulating the ATP7B-H1069Q interactome was proposed as a rescue strategy but specific binding partners that might be targeted for mutant correction remain elusive. Here we try to identify a mutant-specific interactor for the pharmacological rescue of ATP7B-H1069Q.
HostingRepository	PRIDE
AnnounceDate	2020-12-11
AnnouncementXML	Submission_2020-12-11_00:41:23.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Roman Polishchuk
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	6x(13)C labeled residue
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2019-12-18 02:12:26	ID requested
⏵ 1	2020-12-11 00:41:24	announced

Concilli M, Petruzzelli R, Parisi S, Catalano F, Sirci F, Napolitano F, Renda M, Galietta LJV, Di Bernardo D, Polishchuk RS, Pharmacoproteomics pinpoints HSP70 interaction for correction of the most frequent Wilson disease-causing mutant of ATP7B. Proc Natl Acad Sci U S A, 117(51):32453-32463(2020) [pubmed]

submitter keyword: ATP7B, Wilson disease, copper

Roman Polishchuk
contact affiliation	Telethon Institute of Genetics and Medicine (TIGEM)
contact email	polish@tigem.it
lab head
Roman Polishchuk
contact affiliation	Telethon Institute of Genetics and Medicine (TIGEM)
contact email	polish@tigem.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/2020/12/PXD016816

PRIDE project URI

• PRIDE
  1. PXD016816
     1. Label: PRIDE project
     2. Name: Interactomes of WT and H1069Q variants of ATP7B