Proteomics

Dataset Information

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Poglut2/3 double knockout in mice results in neonatal lethality with reduced levels of fibrillin in lung tissues


ABSTRACT: Fibrillins, which are extensively O-glucosylated by POGLUT2 and 3, are crucial for development and cardiopulmonary function. Using primary dermal fibroblasts derived from our POGLUT 2 and 3 double knockout mouse model combined with proteomics analyses, we show that loss of O-glucose modifications on fibrillin causes compositional changes in the extracellular environment compared to controls. Notably, a 30-50% reduction in the abundance of FBN1 and 2 was found in media samples whereas an 85% reduction was found in the extracellular matrix. Other proteins in the extracellular matrix also exhibited changes in abundance in the double knockout samples compared to wild type, but it is still unclear if these changes are simply secondary to the significant reduction in fibrillins.

INSTRUMENT(S): Q Exactive

ORGANISM(S): Mus Musculus (mouse)

TISSUE(S): Primary Cell, Fibroblast

SUBMITTER: Daniel Williamson  

LAB HEAD: Robert S.

PROVIDER: PXD043730 | Pride | 2024-06-10

REPOSITORIES: Pride

Dataset's files

Source:
Action DRS
6-7-23_DermalSecretome.pdResult Other
6-7-23_Dermal_Secretome.msf Msf
DW012122_012522_5.raw Raw
DW012122_1_20220124120307.raw Raw
DW012122_2_20220124151321.raw Raw
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Publications

Poglut2/3 double knockout in mice results in neonatal lethality with reduced levels of fibrillin in lung tissues.

Neupane Sanjiv S   Williamson Daniel B DB   Roth Robyn A RA   Halabi Carmen M CM   Haltiwanger Robert S RS   Holdener Bernadette C BC  

The Journal of biological chemistry 20240604 7


Fibrillin microfibrils play a critical role in the formation of elastic fibers, tissue/organ development, and cardiopulmonary function. These microfibrils not only provide structural support and flexibility to tissues, but they also regulate growth factor signaling through a plethora of microfibril-binding proteins in the extracellular space. Mutations in fibrillins are associated with human diseases affecting cardiovascular, pulmonary, skeletal, and ocular systems. Fibrillins consist of up to 4  ...[more]

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