PXD050486-1

PXD050486 is an original dataset announced via ProteomeXchange.

Title	Loss of the Mitochondrial SAM Transporter Reveals a Lipoylation-Dependent Metabolic Vulnerability in the Postnatal Heart
Description	SLC25A26 encodes the sole mitochondrial S-adenosylmethionine carrier (SAMC). Inside mitochondria, SAM functions as a methyl group donor for various methyltransferases and as a substrate for radical SAM enzymes. Variants in SAMC are associated with mitochondrial disease, and we previously demonstrated that the mitochondrial methylation potential links the one-carbon pool to energy metabolism, but tissue-specific responses are poorly understood. Here, we studied the physiological, molecular, and metabolic consequences of a SAMC deficiency in heart or skeletal muscle. While SAMC ablation in skeletal muscle causes a progressive decline resembling other mitochondrial myopathies, cardiac-specific deletion causes a severe and sudden cardiac phenotype driven by a rapid loss of lipoylation of 2-oxoacid dehydrogenases. This collapse coincides with the transition from milk to solid food, causing a natural reduction in medium-chain fatty acids. We demonstrate that octanoic acid becomes rate-limiting during this transition, exposing a critical requirement for lipoic acid during cardiac maturation.
HostingRepository	PRIDE
AnnounceDate	2026-07-03
AnnouncementXML	Submission_2026-07-03_02:27:31.262.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Akos Vegvari
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: NEWT:10090;
ModificationList	iodoacetamide derivatized residue
Instrument	Q Exactive HF

Revision	Datetime	Status	ChangeLog Entry
0	2024-03-08 07:28:31	ID requested
⏵ 1	2026-07-03 02:27:31	announced

Dataset with its publication pending

submitter keyword: None

Akos Vegvari
contact affiliation	Karolinska Institutet
contact email	akos.vegvari@ki.se
lab head
Akos Vegvari
contact affiliation	Karolinska Institutet
contact email	akos.vegvari@ki.se
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/2026/07/PXD050486

PRIDE project URI

• PRIDE
  1. PXD050486
     1. Label: PRIDE project
     2. Name: Loss of the Mitochondrial SAM Transporter Reveals a Lipoylation-Dependent Metabolic Vulnerability in the Postnatal Heart