Matches in SemOpenAlex for { <https://semopenalex.org/work/W2028159558> ?p ?o ?g. }
- W2028159558 endingPage "61" @default.
- W2028159558 startingPage "47" @default.
- W2028159558 abstract "Congenital muscular dystrophies (CMDs) with associated brain abnormalities are a group of disorders characterized by muscular dystrophy and brain and eye abnormalities that are frequently caused by mutations in known or putative glycotransferases involved in protein O-mannosyl glycosylation. Previous work identified α-dystroglycan as the major substrate for O-mannosylation and its altered glycosylation the major cause of these disorders. However, work from several labs indicated that other proteins in the brain are also O-mannosylated and therefore could contribute to CMD pathology in patients with mutations in the protein O-mannosylation pathway, however few of these proteins have been identified and fully characterized in CMDs. In this study we identify receptor protein tyrosine phosphatase ζ (RPTPζ) and its secreted variant, phosphacan, as another potentially important substrate for protein O-mannosylation in the brain. Using a mouse model of muscle-eye-brain disease lacking functional protein O-mannose β-1,2-N-acetylglucosaminyltransferase (POMGnT1), we show that RPTPζ/phosphacan is shifted to a lower molecular weight and distinct carbohydrate epitopes normally detected on the protein are either absent or substantially reduced, including Human Natural Killer-1 (HNK-1) reactivity. The spatial and temporal expression patterns of these O-mannosylated forms of RPTPζ/phosphacan and its hypoglycosylation and loss of HNK-1 glycan epitopes in POMGnT1 knockouts are suggestive of a role in the neural phenotypes observed in patients and animal models of CMDs." @default.
- W2028159558 created "2016-06-24" @default.
- W2028159558 creator A5003238248 @default.
- W2028159558 creator A5011792766 @default.
- W2028159558 creator A5020753801 @default.
- W2028159558 creator A5042791360 @default.
- W2028159558 date "2012-09-01" @default.
- W2028159558 modified "2023-10-08" @default.
- W2028159558 title "RPTPζ/phosphacan is abnormally glycosylated in a model of muscle–eye–brain disease lacking functional POMGnT1" @default.
- W2028159558 cites W1483108553 @default.
- W2028159558 cites W1502661652 @default.
- W2028159558 cites W1516087394 @default.
- W2028159558 cites W1572408887 @default.
- W2028159558 cites W1599255496 @default.
- W2028159558 cites W1825113405 @default.
- W2028159558 cites W1883330555 @default.
- W2028159558 cites W1963757547 @default.
- W2028159558 cites W1964463588 @default.
- W2028159558 cites W1965241231 @default.
- W2028159558 cites W1966675930 @default.
- W2028159558 cites W1966717150 @default.
- W2028159558 cites W1967481695 @default.
- W2028159558 cites W1968666078 @default.
- W2028159558 cites W1969570221 @default.
- W2028159558 cites W1969832217 @default.
- W2028159558 cites W1970540560 @default.
- W2028159558 cites W1972695095 @default.
- W2028159558 cites W1981447099 @default.
- W2028159558 cites W1981557699 @default.
- W2028159558 cites W1984304291 @default.
- W2028159558 cites W1985654472 @default.
- W2028159558 cites W1985921783 @default.
- W2028159558 cites W1997720118 @default.
- W2028159558 cites W1997925937 @default.
- W2028159558 cites W1998880226 @default.
- W2028159558 cites W2003134522 @default.
- W2028159558 cites W2003726233 @default.
- W2028159558 cites W2007100262 @default.
- W2028159558 cites W2007580007 @default.
- W2028159558 cites W2008115339 @default.
- W2028159558 cites W2008833078 @default.
- W2028159558 cites W2012496453 @default.
- W2028159558 cites W2015598028 @default.
- W2028159558 cites W2018029590 @default.
- W2028159558 cites W2018087626 @default.
- W2028159558 cites W2018177140 @default.
- W2028159558 cites W2026310730 @default.
- W2028159558 cites W2028519929 @default.
- W2028159558 cites W2028799863 @default.
- W2028159558 cites W2030973462 @default.
- W2028159558 cites W2031523308 @default.
- W2028159558 cites W2034163119 @default.
- W2028159558 cites W2034477126 @default.
- W2028159558 cites W2040718007 @default.
- W2028159558 cites W2044593069 @default.
- W2028159558 cites W2046675022 @default.
- W2028159558 cites W2047850625 @default.
- W2028159558 cites W2050274110 @default.
- W2028159558 cites W2060919061 @default.
- W2028159558 cites W2061697489 @default.
- W2028159558 cites W2064758161 @default.
- W2028159558 cites W2065497335 @default.
- W2028159558 cites W2065551216 @default.
- W2028159558 cites W2068690566 @default.
- W2028159558 cites W2068909778 @default.
- W2028159558 cites W2070058093 @default.
- W2028159558 cites W2072491010 @default.
- W2028159558 cites W2073006349 @default.
- W2028159558 cites W2077335072 @default.
- W2028159558 cites W2077411549 @default.
- W2028159558 cites W2078383027 @default.
- W2028159558 cites W2081869444 @default.
- W2028159558 cites W2083273192 @default.
- W2028159558 cites W2085209794 @default.
- W2028159558 cites W2086234005 @default.
- W2028159558 cites W2091832812 @default.
- W2028159558 cites W2095533841 @default.
- W2028159558 cites W2109507582 @default.
- W2028159558 cites W2111591442 @default.
- W2028159558 cites W2120186017 @default.
- W2028159558 cites W2121360720 @default.
- W2028159558 cites W2122230709 @default.
- W2028159558 cites W2124032562 @default.
- W2028159558 cites W2133843400 @default.
- W2028159558 cites W2135383773 @default.
- W2028159558 cites W2138530343 @default.
- W2028159558 cites W2143467209 @default.
- W2028159558 cites W2152980841 @default.
- W2028159558 cites W2153181528 @default.
- W2028159558 cites W2156863768 @default.
- W2028159558 cites W2165945072 @default.
- W2028159558 cites W2167064657 @default.
- W2028159558 doi "https://doi.org/10.1016/j.neuroscience.2012.06.026" @default.
- W2028159558 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3412926" @default.
- W2028159558 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/22728091" @default.
- W2028159558 hasPublicationYear "2012" @default.
- W2028159558 type Work @default.
- W2028159558 sameAs 2028159558 @default.