PXD051963-1

PXD051963 is an original dataset announced via ProteomeXchange.

Title	Effect of Mavacamten on skeletal muscle fibre proteome in nemaline myopathy
Description	Nemaline myopathy (NM) is a genetic muscle disorder, notably caused by mutations in the NEB gene (NEB-NM). Here we investigated the efficacy of a four-week Mavacamten (myosin ATPase inhibitor) treatment using a NEB-NM mouse model. After the four weeks, soleus muscles were extracted, muscle fibres were isolated, and a global untargeted proteomics approach was employed. As presented in the data set, a lot of various proteins were affected by the treatment.
HostingRepository	PRIDE
AnnounceDate	2025-10-14
AnnouncementXML	Submission_2025-10-14_03:37:32.746.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Michael Wierer
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: NEWT:10090;
ModificationList	monohydroxylated residue; iodoacetamide derivatized residue
Instrument	timsTOF Pro 2

Revision	Datetime	Status	ChangeLog Entry
0	2024-05-03 05:29:38	ID requested
⏵ 1	2025-10-14 03:37:33	announced

Laitila J, Seaborne RAE, Ranu N, Kolb JS, Wallgren-Pettersson C, Witting N, Vissing J, Vilchez JJ, Zanoteli E, Palmio J, Huovinen S, Granzier H, Ochala J, Myosin ATPase inhibition fails to rescue the metabolically dysregulated proteome of nebulin-deficient muscle. J Physiol, 602(20):5229-5245(2024) [pubmed]

10.1113/jp286870;

submitter keyword: skeletal muscle cell

nemaline myopathy

nebulin

Mavacamten

Julien Ochala
contact affiliation	University of Copenhagen Department of Biomedical Sciences Blegdamsvej 3B København N, DK-2200
contact email	julien.ochala@sund.ku.dk
lab head
Michael Wierer
contact affiliation	Proteomics Research Infrastructure, University of Copenhagen
contact email	michael.wierer@sund.ku.dk
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/2025/10/PXD051963

PRIDE project URI

• PRIDE
  1. PXD051963
     1. Label: PRIDE project
     2. Name: Effect of Mavacamten on skeletal muscle fibre proteome in nemaline myopathy