PXD006377

PXD006377 is an original dataset announced via ProteomeXchange.

Title	Lack of glycogenin causes glycogen accumulation and muscle function impairment
Description	Glycogenin is considered essential for glycogen synthesis as it acts as a primer for the initiation of the polysaccharide. In this study, we challenge this notion and demonstrate that glycogen can be synthesized in vivo in the absence of glycogenin. Glycogenin-deficient mice (Gyg KO) accumulate high amounts of the polysaccharide in skeletal and cardiac striated muscles. This glycogen shows no covalently bound protein, thereby indicating that no protein primer is essential for glycogen synthesis. Gyg KO mice show lower resting energy expenditure and lesser resistance when subjected to endurance exercise than control animals, which can be attributed to a switch of oxidative myofibers toward glycolytic metabolism. This switch is caused by the over-accumulation of glycogen, since mice overexpressing glycogen synthase specifically in skeletal muscle show a similar metabolic alteration. These results may explain the muscular defects of GSD XV patients, who show high glycogen accumulation in striated muscles.
HostingRepository	PRIDE
AnnounceDate	2017-07-10
AnnouncementXML	Submission_2017-07-11_04:57:20.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Marta Vilaseca
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	hexosylated residue
Instrument	LTQ FT

Revision	Datetime	Status	ChangeLog Entry
0	2017-04-25 05:55:05	ID requested
1	2017-07-06 05:46:33	announced
2	2017-07-10 00:24:13	announced	Updated project metadata.
3	2017-07-10 00:26:49	announced	Updated project metadata.
⏵ 4	2017-07-11 04:57:21	announced	Updated project metadata.

Testoni G, Duran J, Garc, í, a-Rocha M, Vilaplana F, Serrano AL, Sebasti, á, n D, L, ó, pez-Soldado I, Sullivan MA, Slebe F, Vilaseca M, Mu, ñ, oz-C, á, noves P, Guinovart JJ, Lack of Glycogenin Causes Glycogen Accumulation and Muscle Function Impairment. Cell Metab, 26(1):256-266.e4(2017) [pubmed]

curator keyword: Biological

submitter keyword: glycogenin

Joan J. Guinovart
contact affiliation	Institute for Research in Biomedicine, IRB Barcelona, The Barcelona Institute of Science and Technology, BIST
contact email	guinovart@irbbarcelona.org
lab head
Marta Vilaseca
contact affiliation	Institute for Research in Biomedicine (IRB Barcelona), The Barcelona Institute of Science and Technology, Baldiri Reixac, 10, 08028 Barcelona, Spain
contact email	marta.vilaseca@irbbarcelona.org
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/2017/07/PXD006377

PRIDE project URI

• PRIDE
  1. PXD006377
     1. Label: PRIDE project
     2. Name: Lack of glycogenin causes glycogen accumulation and muscle function impairment