PXD021021

PXD021021 is an original dataset announced via ProteomeXchange.

Title	Altered turnover of the CNS myelin protein Opalin in a mouse model of hereditary spastic paraplegia 35
Description	Fatty 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 central 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).
HostingRepository	PRIDE
AnnounceDate	2021-03-04
AnnouncementXML	Submission_2021-03-04_13:44:57.042.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Robert Hardt
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	S-carboxamidoethyl-L-cysteine; monohydroxylated residue; acetylated residue
Instrument	LTQ Orbitrap Velos

Revision	Datetime	Status	ChangeLog Entry
0	2020-08-20 04:08:38	ID requested
⏵ 1	2021-03-04 13:44:57	announced

Hardt R, Jordans S, Winter D, Gieselmann V, Wang-Eckhardt L, Eckhardt M, Decreased turnover of the CNS myelin protein Opalin in a mouse model of hereditary spastic paraplegia 35. Hum Mol Genet, 29(22):3616-3630(2021) [pubmed]

submitter keyword: Isobaric labeling, TMT, myelin, CNS, mice, fatty acid hydroxylation, sphingolipids, fatty acid 2-hydroxylase, FA2H, neurodegeneration, disease model, time course, hereditary spastic paraplegia 35, Opalin

Matthias Eckhardt
contact affiliation	AK Gieselmann Institut für Biochemie und Molekularbiologie (IBMB) Med. Fak. Universtität Bonn Nussallee 11 53115 Bonn Germany
contact email	eckhardt@uni-bonn.de
lab head
Robert Hardt
contact affiliation	IBMB - University of Bonn
contact email	rhardt@uni-bonn.de
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD021021
     1. Label: PRIDE project
     2. Name: Altered turnover of the CNS myelin protein Opalin in a mouse model of hereditary spastic paraplegia 35