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PXD030244

PXD030244 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleAge-dependent increase in Schmidt-Lanterman incisures and a Cadm4 associated membrane skeletal complex in fatty acid 2-hydroxylase deficient mice, a mouse model of spastic paraplegia SPG35
DescriptionFatty acid 2-hydroxylase (FA2H) is responsible for the synthesis of 2-hydroxylated fatty acids, which are a building block of sphingolipids. This 2-hydroxylated sphingolipids can be found in various tissues such as brain, spinal cord, skin, testis, ovary, kidney, stomach, or intestine with a notably high occurrence in the insulating myelin sheath of neurons. Mutations in the FA2H gene can cause the neurodegenerative disease spastic paraplegia 35 (SPG35), which is also known as fatty acid hydroxylase-associated neurodegeneration. Moreover, FA2H knock-out mice largely resemble the SPG35 human disease phenotype. To further elucidate the connection between FA2H and SPG35, we performed a comparative quantitative proteome analysis of peripheral nervous system myelin of wildtype and FA2H-KO mice at different ages (6, 13, and 17 months) using isobaric labeling with tandem mass tags (TMT).
HostingRepositoryPRIDE
AnnounceDate2022-04-22
AnnouncementXMLSubmission_2022-04-22_05:48:18.624.xml
DigitalObjectIdentifierhttps://dx.doi.org/10.6019/PXD030244
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportSupported dataset by repository
PrimarySubmitterRobert Hardt
SpeciesList scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationListTMT6plex-126 reporter+balance reagent acylated residue; S-carboxamidoethyl-L-cysteine; monohydroxylated residue; acetylated residue
InstrumentLTQ Orbitrap Velos
Dataset History
RevisionDatetimeStatusChangeLog Entry
02021-12-08 07:32:37ID requested
12022-04-22 05:48:18announced
Publication List
10.1007/S12035-022-02832-4;
Keyword List
submitter keyword: Isobaric labelling, TMT, myelin, PNS, mice, fatty acid hydroxylation, sphingolipids, fatty acid 2-hydroxylase, FA2H, neurodegeneration, disease model, time course, hereditary spastic paraplegia type 35, Schmidt-Lanterman incisures, sciatic nerves
Contact List
Matthias Eckhardt
contact affiliationAK Becker, Institut für Biochemie und Molekularbiologie (IBMB), Med. Fak. Universtität Bonn, Nussallee 11, 53115 Bonn, Germany
contact emaileckhardt@uni-bonn.de
lab head
Robert Hardt
contact affiliationInstitute for Biochemistry and Molecular Biology, Medical Faculty, University of Bonn, 53115 Bonn, Germany
contact emailrhardt@uni-bonn.de
dataset submitter
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Dataset FTP location
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