PXD037129

PXD037129 is an original dataset announced via ProteomeXchange.

Title	Impact of mannosylation on structure and functionality of ER and Golgi
Description	Many proteins undergo glycosylation in the endoplasmic reticulum (ER) and the Golgi apparatus. Altered glycosylation can manifest in serious, sometimes fatal malfunctions. We recently showed that mutations in the cytoplasmic protein GDP-mannose pyrophosphorylase A (GMPPA) cause a syndrome characterized by alacrima, achalasia, mental retardation and myopathic alterations. GMPPA acts as feedback inhibitor of GDP-mannose pyrophosphorylase B (GMPPB), which provides GDP-mannose as a substrate for protein glycosylation. Loss of GMPPA enhances incorporation of mannose into glycochains of various proteins, including α-dystroglycan (α-DG), a protein that links the extracellular matrix with the cytoskeleton. Here, we show that loss of GMPPA affects the functionality of the Golgi apparatus using different approaches. First, we show a fragmentation of the Golgi apparatus in skeletal muscle fibers and in neurons of GMPPA KO mice. A major reorganization is also evident by mass spectrometry of KO tissues with a regulation of several ER- and Golgi-resident proteins. We further show that loss of GMPPA increases the retention of α-DG in the ER. Notably, mannose supplementation can mimic changes in ER and Golgi structure and function in WT cells. In summary, our data underline the importance of a balanced mannose homeostasis for structure and function of the secretory pathway.
HostingRepository	PRIDE
AnnounceDate	2023-11-14
AnnouncementXML	Submission_2023-11-14_08:37:31.366.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Emilio Cirri
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	No PTMs are included in the dataset
Instrument	Orbitrap Fusion Lumos

Revision	Datetime	Status	ChangeLog Entry
0	2022-10-02 03:56:03	ID requested
1	2023-03-10 22:12:30	announced
⏵ 2	2023-11-14 08:37:32	announced	2023-11-14: Updated project metadata.

Franzka P, Sch, ü, ler SC, Kentache T, Storm R, Bock A, Katona I, Weis J, Buder K, Kaether C, H, ü, bner CA, Impact of Hypermannosylation on the Structure and Functionality of the ER and the Golgi Complex. Biomedicines, 11(1):(2023) [pubmed]

submitter keyword: golgi network

endoplasmic reticulum

mannosylation

Christian A. Hübner
contact affiliation	Institute of Human Genetics, University Hospital Jena, Germany
contact email	christian.huebner@med.uni-jena.de
lab head
Emilio Cirri
contact affiliation	Leibniz Institute on Ageing Fritz Lipmann Institute Jena
contact email	emilio.cirri@fli-leibniz.de
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD037129
     1. Label: PRIDE project
     2. Name: Impact of mannosylation on structure and functionality of ER and Golgi