Project description:To identify brain region specific proteins that contribute to brain region specific neurodegeneration, RNA sequencing was performed using cerebellum, striatum and prefrontal cortex tissues from 3-month-old SCA17 knock-in (KI) mice and wild-type (WT) littermates. We find 245 cerebellar specifically dysregulated genes (110 up-regulated genes and 145 down-regulated genes), 133 striatum specifically dysregulated genes (45 up-regulated genes and 88 down-regulated genes) and 17 prefrontal cortex specifically dysregulated genes (4 up-regulated genes and 13 down-regulated genes). Combined with other evidences, we demonstrate that cerebellum-enriched protein INPP5A contributes to selective neuropathology in mouse model of Spinocerebellar ataxias type 17
Project description:Purpose:Next-generation sequencing has revolutionized sytems-level celluar pathway analysis. The goals of this study are to compare the U87 cell xenograft GBM mice (U87 cell line) to TWIST1 knock out U87 cell xenograft GBM mice (TWIST1 knock out U87 cell line) using their transcriptomes
Project description:We investigated brain tissue from N471D WASH complex subunit strumpellin knock-in mice as a genetic model for hereditary spastic paraplegia type 8. While WASHC5-related protein interaction partners and complexes showed no change in abundancies, the proteomic analysis depicted consistent upregulation of BPTF and downregulation of KLHL11 in heterozygous and homozygous knock-in mice. This finding suggests mechanistic links for hereditary spastic paraplegia type 8 through the roles of BPTF and KLHL11 in neurodegeneration and protein quality control, respectively.
Project description:Ewing’s Sacroma (EWS) is a carcinogenic bone tumor which is predominantly occurred in young adults. EWS RNA binding protein 1 (Ewsr1) protein is well-known as a multifunctional protein and its epigenetic signatures contribute to the pathogenesis of various human disease such as neurodegenerative disorders, skin development, or tumorigenic process. However, the specific cellular function and physiological characteristics of Ewsr1 are still ambiguous. In this study, therefore, we applied TMT-labeled quantitative mass spectrometry-based proteomic approach to investigate the global proteome changes of brain tissue between Ewsr1 knock-out (KO) and wild type (WT) mice (n=3, each).
Project description:The ubiquitin ligase Peli1 has previously been suggested as a potential treatment target in multiple sclerosis since the knock-out induced less activated microglia and less inflammation in the CNS of experimental autoimmune encephalomyelitis in mice. In the present study the brain proteomes of Peli1 knock-out mice and wild-type mice were analyzed and compared before disease induction and after 10 and 20 days of experimental autoimmune encephalomyelitis using quantitative proteomics. The brain samples were analyzed using TMT labeling of small pools of samples and verified using label-free of individual mice.