期刊
BIOMOLECULAR NMR ASSIGNMENTS
卷 8, 期 2, 页码 325-327出版社
SPRINGER
DOI: 10.1007/s12104-013-9509-z
关键词
AXH; SCA1; Poly-glutamine; Conformational switch; CIC; Transcriptional repressor
资金
- Medical Research Council [MC_PC_13054, MC_U117533887, MC_U117584256] Funding Source: Medline
- MRC [MC_U117584256, MC_U117533887, MC_PC_13054] Funding Source: UKRI
- Medical Research Council [MC_U117533887, MC_PC_13054, MC_U117584256] Funding Source: researchfish
Ataxin-1 is the protein responsible for the genetically-inherited neurodegenerative disease spinocerebellar ataxia type-1 linked to the expansion of a polyglutamine tract within the protein sequence. The AXH domain of ataxin-1 is essential for the protein to function as a transcriptional co-repressor and mediates the majority of the interactions of ataxin-1 with cellular partners, mainly transcriptional regulators. One of the best characterized ataxin-1 functional partners is Capicua (CIC), a transcriptional repressor involved in signalling pathways that regulate mammalian development, tumorigenesis and, through the interaction with ataxin-1, also neurodegeneration. Complex formation of ataxin-1 with CIC is important both for the function of the wild-type protein and for pathogenesis as transcriptional disregulation is observed since the early stages of the development of the disease. Here we report the H-1, C-13 and N-15 backbone and side-chain chemical shift assignments of the human ataxin-1 AXH domain in complex with a CIC ligand-peptide.
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