PXD078106

PXD078106 is an original dataset announced via ProteomeXchange.

Title	Tetrahedral DNA Nanostructure-delivered Suppressor tRNA Ameliorates Nephropathy in COL4A5 Nonsense Mutation-mediated Alport Syndrome Mice
Description	Nonsense mutations introduce premature termination codons (PTCs) that trigger mRNA degradation and underlie genetic diseases, including Alport syndrome (AS). Suppressor tRNAs (sup-tRNAs) are potential therapies for such disorders, as they can precisely readthrough PTCs and restore full-length protein synthesis. For AS caused by COL4A5 nonsense-mediated deficiency, we developed a TDN-sup-tRNA delivery strategy using tetrahedral DNA nanostructures (TDNs) characterized by excellent biocompatibility and renal targeting. In a Col4a5-R471X mouse model, TDN-sup-tRNA achieved high readthrough efficiency, restoring COL4A5 protein expression and reconstituting glomerular basement membrane integrity. Notably, proteinuria was reduced to 35% of baseline within four weeks. Compared with AAV2/9-mediated delivery, this system exhibited enhanced renal targeting and superior therapeutic efficacy over a one-month treatment period, without detectable toxicity. Collectively, this strategy represents a safe and effective non-viral RNA therapeutic approach for precise correction of nonsense mutation–associated renal diseases.
HostingRepository	PRIDE
AnnounceDate	2026-05-13
AnnouncementXML	Submission_2026-05-13_07:10:26.225.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Dan Hong
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: NEWT:9606;
ModificationList	No PTMs are included in the dataset
Instrument	Orbitrap Fusion Lumos

Revision	Datetime	Status	ChangeLog Entry
0	2026-05-07 02:56:21	ID requested
⏵ 1	2026-05-13 07:10:26	announced

Dataset with its publication pending

submitter keyword: COL4A5,suppressor tRNA

Qing Xia
contact affiliation	State Key Laboratory of Natural and Biomimetic Drugs, Department of Molecular and Cellular Pharmacology, School of Pharmaceutical Sciences, Peking University, Beijing 100191, China.
contact email	xsqing@hsc.pku.edu.cn
lab head
Dan Hong
contact affiliation	Peking University
contact email	2210307314@stu.pku.edu.cn
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/2026/05/PXD078106

PRIDE project URI

• PRIDE
  1. PXD078106
     1. Label: PRIDE project
     2. Name: Tetrahedral DNA Nanostructure-delivered Suppressor tRNA Ameliorates Nephropathy in COL4A5 Nonsense Mutation-mediated Alport Syndrome Mice