Proteomics

Dataset Information

0

LC-MS/MS analysis of Mannose 6-phosphate containing proteins


ABSTRACT: Mucolipidosis III gamma (MLIII) is clinically characterized by onset of first symptoms at an average of 5 years such as stiffness of hands and shoulders, claw hand deformities, scoliosis and progressive destruction of hip joints. The disease is caused by mutations in GNPTG encoding the gamma-subunit of the GlcNAc-1-phosphotransferase complex. This enzyme is responsible for the generation of mannose 6-phosphate (M6P) targeting signals on 70 soluble lysosomal enzymes that are required for their efficient receptor-mediated transport to lysosomes. In fibroblasts from Gnptg-KO mice the GlcNAc-1-phosphotransferase activity is reduced leading to low amounts of M6P-containing proteins. M6P affinity chromatography-assisted and mass spectrometry-based secretome analysis of Gnptg-KO fibroblasts revealed a distinct set of lysosomal enzymes modified with M6P residues.

INSTRUMENT(S): LTQ Orbitrap Velos

ORGANISM(S): Mus Musculus (mouse)

TISSUE(S): Fibroblast

DISEASE(S): Disease Free

SUBMITTER: Shiva Ahmadi  

LAB HEAD: Dominic Winter

PROVIDER: PXD007546 | Pride | 2018-06-06

REPOSITORIES: Pride

Dataset's files

Source:
Action DRS
MaxQuantTxtFiles.zip Other
SA_HH_Bead1.raw Raw
SA_HH_Bead2.raw Raw
SA_HH_Bead3.raw Raw
evidence.txt Txt
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