⮝ Full datasets listing
PXD060094
PXD060094 is an original dataset announced via ProteomeXchange.
Dataset Summary
| Title | Deciphering Jointly Dysregulated Pathways in Liver and Motor Neurons of Spinal Muscular Atrophy: A Study using Patient-Derived iPSCs |
| Description | Spinal Muscular Atrophy (SMA), primarily known as a motor neuron disease, exhibits significant extra-neuronal phenotypes. The liver is one such extra-neuronal organ critically implicated but little is known of the overlapping biological pathways that could be jointly dysregulated in the liver and motor neurons. This study aims to bridge this gap by utilizing SMA patient-derived induced hepatocytes (iHeps) and induced motor neurons (iMNs) for proteomics and gene ontologygene enrichment analyses. CRISPR-edited isogenic wild type and carrier lines were also used to show SMN dependency. Following proteomics and gene ontology gene enrichment analysis, a thorough PubMed literature review was conducted, which suggested potential causal or compensatory roles in various protein hits that were concordantly dysregulated in both SMA iHeps and iMNs. Comparative analysis between SMA iHep and iMN proteome profiles also revealed distinct differences, highlighting unique tissue-specific pathology. Additionally, connectivity map analysis identified drug candidates that could potentially target both SMA iHeps and iMNs, based on the concordantly dysregulated proteins that returned sufficient literature results for causal or compensatory roles in SMA. Overall, this study provides greater understanding of how hepatocyte dysfunction interacts with motor neuron pathology at the molecular basis in SMA. |
| HostingRepository | jPOST |
| AnnounceDate | 2026-01-23 |
| AnnouncementXML | Submission_2026-01-22_07:00:05.226.xml |
| DigitalObjectIdentifier | |
| ReviewLevel | Non peer-reviewed dataset |
| DatasetOrigin | Original dataset |
| RepositorySupport | Unsupported dataset by repository |
| PrimarySubmitter | Radoslaw Sobota |
| SpeciesList | scientific name: Homo sapiens (Human); NCBI TaxID: 9606; |
| ModificationList | S-carboxamidomethyl-L-cysteine; L-methionine sulfoxide; alpha-amino acetylated residue; deamidated L-glutamine; deamidated L-asparagine |
| Instrument | instrument |
Dataset History
| Revision | Datetime | Status | ChangeLog Entry |
|---|---|---|---|
| 0 | 2025-01-22 22:17:27 | ID requested | |
| ⏵ 1 | 2026-01-22 07:00:05 | announced |
Publication List
| Dataset with its publication pending |
Keyword List
| submitter keyword: Hepatocytes, Motor Neurons, Spinal Muscular Atrophy |
Contact List
| Crystal Yeo Jing Jing | |
|---|---|
| lab head | |
| Radoslaw Sobota | |
| contact affiliation | IMCB A-STAR Singapore |
| dataset submitter | |
Full Dataset Link List
| jPOST dataset URI |
| 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.jpostdb.org/JPST003562/ |
