PXD030970 is an
original dataset announced via ProteomeXchange.
Dataset Summary
Title | Mass spectrometry-based proteomic comparison of BirA-SMN, BirA-SMN-Y109C and BirA proxisomes. |
Description | Although recent advances in gene therapy provide hope for spinal muscular atrophy (SMA) patients, the pathology remains the leading genetic cause of infant mortality. SMA is a monogenic pathology that originates from the loss of the SMN1 gene in most cases or mutations in rare cases. Interestingly, SMN1 mutations occur broadly either within the TUDOR methyl-arginine (Rme) reader domain of SMN or its carboxy terminal oligomerization domain. We hypothesized that in SMN1 mutant cases, SMA may emerge from aberrant protein-protein interactions between SMN and key neuronal factors. Using a proximity-dependent biotinylation proteomic (BioID) approach, we have identified and validated a number of SMN-interacting proteins, including Fragile X mental retardation family members (FMRFM), such as FMRP itself as well as FXR1 and FXR2, some depending on a wild-type version of the TUDOR domain. |
HostingRepository | PRIDE |
AnnounceDate | 2023-11-14 |
AnnouncementXML | Submission_2023-11-14_07:55:32.576.xml |
DigitalObjectIdentifier | https://dx.doi.org/10.6019/PXD030970 |
ReviewLevel | Peer-reviewed dataset |
DatasetOrigin | Original dataset |
RepositorySupport | Supported dataset by repository |
PrimarySubmitter | Yohann Couté |
SpeciesList | scientific name: Homo sapiens (Human); NCBI TaxID: 9606; |
ModificationList | biotinylated residue; acetylated residue; monohydroxylated residue; iodoacetamide derivatized residue |
Instrument | Q Exactive HF |
Dataset History
Revision | Datetime | Status | ChangeLog Entry |
0 | 2022-01-14 08:26:36 | ID requested | |
1 | 2022-10-19 03:08:24 | announced | |
⏵ 2 | 2023-11-14 07:55:33 | announced | 2023-11-14: Updated project metadata. |
Publication List
Binda O, Juillard F, Ducassou JN, Kleijwegt C, Paris G, Didillon A, Baklouti F, Corpet A, Cout, é Y, C, ô, t, é J, Lomonte P, SMA-linked SMN mutants prevent phase separation properties and SMN interactions with FMRP family members. Life Sci Alliance, 6(1):(2023) [pubmed] |
Keyword List
submitter keyword: Survival Motor Neuron (SMN),Spinal Muscle Atrophy (SMA), BioID, nanoLC-MS/MS |
Contact List
Yohann Couté |
contact affiliation | EDyP |
contact email | yohann.coute@cea.fr |
lab head | |
Yohann Couté |
contact affiliation | EDyP |
contact email | yohann.coute@cea.fr |
dataset submitter | |
Full Dataset Link List
Dataset FTP location
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PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD030970
- Label: PRIDE project
- Name: Mass spectrometry-based proteomic comparison of BirA-SMN, BirA-SMN-Y109C and BirA proxisomes.