PXD015320-2

PXD015320 is an original dataset announced via ProteomeXchange.

Title	Proteostasis network alteration in lysosomal storage disorders: insights from the mouse model of Krabbe diseaseProteostasis network alteration in lysosomal storage disorders: insights from the mouse model of Krabbe diseaseProteostasis network alteration in lysosomal storage disorders: insights from the mouse model of Krabbe disease
Description	Krabbe disease is characterized by widespread demyelination due to cell death by apoptosis, mainly in oligodendrocytes and Schwann cells. In this study, we highlight that in the brain of 6 days old twitcher mice (model of Krabbe disease) there is evidence of impairment in protein synthesis and degradation, as well as in cytoskeleton remodeling, with a lamin meshwork disaggregation and clear evidence of apoptosis, also demonstrated in oligodendrocytes. Even if further studies are needed to address the mechanisms behind this pathology, this study provide interesting insights on the early onset of Krabbe disease that may be useful for developing of innovative pharmacological treatment.
HostingRepository	PRIDE
AnnounceDate	2024-10-22
AnnouncementXML	Submission_2024-10-22_04:57:11.435.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Claudia Landi
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	No PTMs are included in the dataset
Instrument	ultraflex

Revision	Datetime	Status	ChangeLog Entry
0	2019-09-04 07:59:34	ID requested
1	2019-12-09 02:24:12	announced
⏵ 2	2024-10-22 04:57:11	announced	2024-10-22: Updated project metadata.

Dataset with its publication pending

submitter keyword: cytoskeleton remodelling, lamin meshwork disaggregation,Krabbe disease, twitcher, ER stress

Luca Bini
contact affiliation	Functional Proteomics Lab. Life Science Dpt. Siena University Via Aldo Moro, 2 53100 Siena Italy
contact email	luca.bini@unisi.it
lab head
Claudia Landi
contact affiliation	University of Siena
contact email	landi35@unisi.it
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/2019/12/PXD015320

PRIDE project URI

• PRIDE
  1. PXD015320
     1. Label: PRIDE project
     2. Name: Proteostasis network alteration in lysosomal storage disorders: insights from the mouse model of Krabbe diseaseProteostasis network alteration in lysosomal storage disorders: insights from the mouse model of Krabbe diseaseProteostasis network alteration in lysosomal storage disorders: insights from the mouse model of Krabbe disease