PXD020403

PXD020403 is an original dataset announced via ProteomeXchange.

Title	Mitochondrial defects contribute to impaired translational in SMA motor neurons
Description	Spinal muscular atrophy (SMA) is a neuromuscular disease, characterized by loss of lower alpha motoneurons, which leads to proximal muscle weakness. SMA is caused by reduced levels of Survival of Motor Neuron protein due to biallelic deletions or mutations in the SMN1 gene. Dysfunctional mitochondria can harm cells by decreased ATP production, but also by increased oxidative stress due to elevated production of reactive oxygen species (ROS). In this study, whole proteome analysis was performed using the Taiwanese SMA mouse model on an FVB/N background to identify changes in the proteome related to oxidative stress. Therefore, primary WT and SMA motoneurons were treated with a known anti-oxidant N-Acetylcysteine, the ROS inducer menadione and the cell culture supplement sodium pyruvate. Furthermore, this study aims to investigate changes in mRNA translation initiation affected by oxidative stress in SMA.
HostingRepository	PRIDE
AnnounceDate	2024-10-22
AnnouncementXML	Submission_2024-10-22_05:17:02.985.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Maximilian Paul Thelen
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	monohydroxylated residue
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2020-07-16 07:27:33	ID requested
1	2020-12-23 01:51:04	announced
2	2021-03-09 23:13:59	announced	2021-03-10: Updated publication reference for PubMed record(s): 33353564.
⏵ 3	2024-10-22 05:17:03	announced	2024-10-22: Updated project metadata.

10.1186/s40478-020-01101-6;

Thelen MP, Wirth B, Kye MJ, Mitochondrial defects in the respiratory complex I contribute to impaired translational initiation via ROS and energy homeostasis in SMA motor neurons. Acta Neuropathol Commun, 8(1):223(2020) [pubmed]

submitter keyword: initiation of mRNA translation, reactive oxygen species, mitochondria, SMN,Spinal muscular atrophy

Min Jeong Kye
contact affiliation	Institute of human genetics, University of cologne
contact email	min.kye@uk-koeln.de
lab head
Maximilian Paul Thelen
contact affiliation	Institute of human genetics, University of Cologne
contact email	maximilian.thelen@uk-koeln.de
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD020403
     1. Label: PRIDE project
     2. Name: Mitochondrial defects contribute to impaired translational in SMA motor neurons