PubMed_ArguminSci  

PubMed:28334871 JSONTXT 6 Projects

Annnotations TAB TSV DIC JSON TextAE

Id Subject Object Predicate Lexical cue
T1 133-293 DRI_Background denotes Mutations in the depalmitoylation enzyme, palmitoyl protein thioesterase (PPT1), result in the early onset neurodegenerative disease known as Infantile Neuronal
T2 315-316 DRI_Background denotes .
T3 317-421 DRI_Approach denotes Here, we provide proteomic evidence suggesting that PPT1 deficiency could be considered as a ciliopathy.
T4 422-606 DRI_Background denotes Analysis of membrane proteins from brain enriched for acylated proteins from neonate Ppt1 knock out and control mice revealed a list of 88 proteins with differential expression levels.
T5 607-703 DRI_Approach denotes Amongst them, we identified Rab3IP, which regulates ciliogenesis in concert with Rab8 and Rab11.
T6 704-773 DRI_Background denotes Immunostaining analysis revealed that PPT1 is localized in the cilia.
T7 774-933 DRI_Background denotes Indeed, an unbiased proteomics analysis on isolated cilia revealed 660 proteins, which differed in their abundance levels between wild type and Ppt1 knock out.
T8 934-1085 DRI_Approach denotes We demonstrate here that Rab3IP, Rab8 and Rab11 are palmitoylated, and that palmitoylation of Rab11 is required for correct intracellular localization.
T9 1086-1281 DRI_Background denotes Cells and brain preparations from Ppt1-/- mice exhibited fewer cells with cilia and abnormally longer cilia, with both acetylated tubulin and Rab3IP wrongly distributed along the length of cilia.
T10 1282-1514 DRI_Outcome denotes Most importantly, the analysis revealed a difference in the distribution and levels of the modified proteins in cilia in the retina of mutant mice versus the wildtype, which may be important in the early neurodegenerative phenotype.
T11 1515-1644 DRI_Outcome denotes Overall, our results suggest a novel link between palmitoylated proteins, cilial organization and the pathophysiology of Neuronal
T12 1666-1667 DRI_Outcome denotes .