PXD068411

PXD068411 is an original dataset announced via ProteomeXchange.

Title	Reduction of charged glycoconjugates in TANGO2 KO mouse brain
Description	The transport and Golgi organization 2 (TANGO2) gene has been identified to cause a rare recessive genetic disorder known as TANGO2-deficiency disorder (TDD). TDD is a multisystemic disorder that can affect different organs including the brain, heart, and muscles, presenting as recurrent metabolic crises, muscle weakness, and neurological symptoms including developmental delay and seizures. In a TANGO2-KO Cal51 cell line model, N-glycomics of the TANGO2-KO cell line showed a significant change in glycomics profile, that was also corroborated with RNAseq transcriptomics showing increased mRNA levels of the respective glycosyltransferases. To investigate the effects of TANGO2-KO on the glycans in the brain of a mouse model, we profiled the polysialic acid (polySia), glycosaminoglycans (GAG), glycolipid glycans (GSL), and protein N- and O-glycans (NG, OG) of the cortex of the TANGO2-KO mice using the Same Sample Sequential Multi-Glycomics workflow. Overall, we observed a severe decrease in chondroitin sulfate GAG disaccharides, and a mild decrease in sialylation across GSL, NG and OG, resulting in a broad decrease in negatively charged glycoconjugates. When comparing against an independent DDA LFQ proteomics acquisition of the same mice model, amongst the glyco-enzymes, only sialic acid synthase (NANS) and chondroitin polymerising factor (CHPF) was also found to be significantly down regulated in the brain, correlating with the changes observed by the multi-glycomics analysis. This overall decrease in negatively charged glycoconjugates due to the loss of TANGO2 function might thus relate to the neurological impairments of TDD.
HostingRepository	PRIDE
AnnounceDate	2026-02-10
AnnouncementXML	Submission_2026-02-09_22:35:18.970.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Timothy McCubbin
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: NEWT:10090;
ModificationList	monohydroxylated residue; iodoacetamide derivatized residue
Instrument	Orbitrap Exploris 480

Revision	Datetime	Status	ChangeLog Entry
0	2025-09-16 07:30:06	ID requested
⏵ 1	2026-02-09 22:35:19	announced

10.1002/PGR2.70042;

submitter keyword: TANGO2, mouse, glycomics, DDA

Nicolle H. Packer
contact affiliation	ARC Centre of Excellence in Synthetic Biology, Macquarie University
contact email	nicki.packer@mq.edu.au
lab head
Timothy McCubbin
contact affiliation	University of Queensland
contact email	t.mccubbin@uq.edu.au
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD068411
     1. Label: PRIDE project
     2. Name: Reduction of charged glycoconjugates in TANGO2 KO mouse brain