Matches in SemOpenAlex for { <https://semopenalex.org/work/W4200365087> ?p ?o ?g. }
- W4200365087 endingPage "35388" @default.
- W4200365087 startingPage "35375" @default.
- W4200365087 abstract "Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disorder characterized by loss of motor neurons (MN) in the spinal cord leading to progressive muscle atrophy and weakness. SMA is caused by mutations in the survival motor neuron 1 (SMN1) gene, resulting in reduced levels of survival motor neuron (SMN) protein. The mechanisms that link SMN deficiency to selective motor neuron dysfunction in SMA remain largely unknown. We present here, for the first time, a comprehensive quantitative TMT-10plex proteomics analysis that covers the development of induced pluripotent stem cell-derived MNs from both healthy individuals and SMA patients. We show that the proteomes of SMA samples segregate from controls already at early stages of neuronal differentiation. The altered proteomic signature in SMA MNs is associated with mRNA splicing, ribonucleoprotein biogenesis, organelle organization, cellular biogenesis, and metabolic processes. We highlight several known SMN-binding partners and evaluate their expression changes during MN differentiation. In addition, we compared our study to human and mouse in vivo proteomic studies revealing distinct and similar signatures. Altogether, our work provides a comprehensive resource of molecular events during early stages of MN differentiation, containing potentially therapeutically interesting protein expression profiles for SMA." @default.
- W4200365087 created "2021-12-31" @default.
- W4200365087 creator A5000797315 @default.
- W4200365087 creator A5002918511 @default.
- W4200365087 creator A5011769862 @default.
- W4200365087 creator A5013176510 @default.
- W4200365087 creator A5044622080 @default.
- W4200365087 creator A5077705713 @default.
- W4200365087 creator A5078920102 @default.
- W4200365087 creator A5080771881 @default.
- W4200365087 date "2021-12-15" @default.
- W4200365087 modified "2023-10-13" @default.
- W4200365087 title "Spinal Muscular Atrophy Patient iPSC-Derived Motor Neurons Display Altered Proteomes at Early Stages of Differentiation" @default.
- W4200365087 cites W1894162067 @default.
- W4200365087 cites W1966660727 @default.
- W4200365087 cites W1971411354 @default.
- W4200365087 cites W1974448552 @default.
- W4200365087 cites W1977374662 @default.
- W4200365087 cites W1977499112 @default.
- W4200365087 cites W1980477780 @default.
- W4200365087 cites W1980905234 @default.
- W4200365087 cites W1987687980 @default.
- W4200365087 cites W1990033911 @default.
- W4200365087 cites W1990393476 @default.
- W4200365087 cites W1992985034 @default.
- W4200365087 cites W1996412713 @default.
- W4200365087 cites W2019293837 @default.
- W4200365087 cites W2021314069 @default.
- W4200365087 cites W2031792886 @default.
- W4200365087 cites W2045433402 @default.
- W4200365087 cites W2047268435 @default.
- W4200365087 cites W2048611034 @default.
- W4200365087 cites W2052064222 @default.
- W4200365087 cites W2054380003 @default.
- W4200365087 cites W2062890651 @default.
- W4200365087 cites W2070007143 @default.
- W4200365087 cites W2070086105 @default.
- W4200365087 cites W2076194625 @default.
- W4200365087 cites W2082707643 @default.
- W4200365087 cites W2087786585 @default.
- W4200365087 cites W2096650232 @default.
- W4200365087 cites W2101499400 @default.
- W4200365087 cites W2108075874 @default.
- W4200365087 cites W2108430210 @default.
- W4200365087 cites W2109273258 @default.
- W4200365087 cites W2112430789 @default.
- W4200365087 cites W2119435334 @default.
- W4200365087 cites W2119729707 @default.
- W4200365087 cites W2124852855 @default.
- W4200365087 cites W2126079277 @default.
- W4200365087 cites W2132341877 @default.
- W4200365087 cites W2135351988 @default.
- W4200365087 cites W2136868631 @default.
- W4200365087 cites W2138977668 @default.
- W4200365087 cites W2148071380 @default.
- W4200365087 cites W2148879151 @default.
- W4200365087 cites W2151093888 @default.
- W4200365087 cites W2153484644 @default.
- W4200365087 cites W2159675211 @default.
- W4200365087 cites W2164748306 @default.
- W4200365087 cites W2165235225 @default.
- W4200365087 cites W2166542841 @default.
- W4200365087 cites W2233211912 @default.
- W4200365087 cites W2309128823 @default.
- W4200365087 cites W2410123768 @default.
- W4200365087 cites W2505641731 @default.
- W4200365087 cites W2539798801 @default.
- W4200365087 cites W2586338189 @default.
- W4200365087 cites W2733659072 @default.
- W4200365087 cites W2734902796 @default.
- W4200365087 cites W2750188054 @default.
- W4200365087 cites W2769295220 @default.
- W4200365087 cites W2770254801 @default.
- W4200365087 cites W2787482106 @default.
- W4200365087 cites W2790608263 @default.
- W4200365087 cites W2803950888 @default.
- W4200365087 cites W2897845597 @default.
- W4200365087 cites W2944790531 @default.
- W4200365087 cites W2949862897 @default.
- W4200365087 cites W2968765313 @default.
- W4200365087 cites W2997022986 @default.
- W4200365087 cites W3021617927 @default.
- W4200365087 cites W3030031815 @default.
- W4200365087 cites W3041597962 @default.
- W4200365087 cites W3087324171 @default.
- W4200365087 cites W3092919613 @default.
- W4200365087 cites W3123624363 @default.
- W4200365087 cites W3196123322 @default.
- W4200365087 cites W4294107304 @default.
- W4200365087 cites W937584289 @default.
- W4200365087 doi "https://doi.org/10.1021/acsomega.1c04688" @default.
- W4200365087 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/34984269" @default.
- W4200365087 hasPublicationYear "2021" @default.
- W4200365087 type Work @default.
- W4200365087 citedByCount "5" @default.
- W4200365087 countsByYear W42003650872022 @default.
- W4200365087 countsByYear W42003650872023 @default.
- W4200365087 crossrefType "journal-article" @default.