PMC:5118426 / 26089-27237 JSONTXT

{"target":"http://pubannotation.org/docs/sourcedb/PMC/sourceid/5118426","sourcedb":"PMC","sourceid":"5118426","source_url":"https://www.ncbi.nlm.nih.gov/pmc/5118426","text":"Spinocerebellar ataxia type 1 (SCA1) is another dominantly inherited neurodegenerative disorder characterized chiefly by progressive ataxia (Meera et al., 2016). SCA1 is caused by expansion of an unstable CAG trinucleotide repeat that encodes a glutamine tract in the Ataxin-1 gene (ATXN1). The disorder is characterized primarily by degeneration of Purkinje neurons of the cerebellum. TUDCA treatment was used to determine whether the survival of Purkinje cells and the onset and progression of ataxia was altered in a transgenic mouse model of SCA1, in which 30 copies of the human ATXN1 cDNA containing a CAG trinucleotide repeat of 82 repeats was under the control of the Pcp-2 Purkinje-cell specific promoter l (Kaemmerer et al., 2001). Despite the successful administration of TUDCA using a similar subcutaneous injection protocol as described above for the R6/2 transgenic HD mouse, and documentation of increased levels in the brain, no effects on cell survival or on the neurological phenotype were noted. The disparate effects in two different genetic models of neurodegenerative disease, suggest that bile acids target specific pathways.","tracks":[{"project":"MyTest","denotations":[{"id":"27920719-27198167-31054363","span":{"begin":155,"end":159},"obj":"27198167"},{"id":"27920719-11274790-31054364","span":{"begin":735,"end":739},"obj":"11274790"}],"namespaces":[{"prefix":"_base","uri":"https://www.uniprot.org/uniprot/testbase"},{"prefix":"UniProtKB","uri":"https://www.uniprot.org/uniprot/"},{"prefix":"uniprot","uri":"https://www.uniprot.org/uniprotkb/"}],"attributes":[{"subj":"27920719-27198167-31054363","pred":"source","obj":"MyTest"},{"subj":"27920719-11274790-31054364","pred":"source","obj":"MyTest"}]},{"project":"2_test","denotations":[{"id":"27920719-27198167-31054363","span":{"begin":155,"end":159},"obj":"27198167"},{"id":"27920719-11274790-31054364","span":{"begin":735,"end":739},"obj":"11274790"}],"attributes":[{"subj":"27920719-27198167-31054363","pred":"source","obj":"2_test"},{"subj":"27920719-11274790-31054364","pred":"source","obj":"2_test"}]}],"config":{"attribute types":[{"pred":"source","value type":"selection","values":[{"id":"MyTest","color":"#b293ec","default":true},{"id":"2_test","color":"#93ec98"}]}]}}