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Construction of a hybrid β-hexosaminidase subunit capable of forming stable homodimers that hydrolyze GM2 ganglioside in vivo
Tay-Sachs or Sandhoff disease result from mutations in either the evolutionarily related HEXA or HEXB genes encoding respectively, the α- or β-subunits of β-hexosaminidase A (HexA). Of the three Hex isozymes, only HexA can interact with its cofactor, the GM2 activator protein (GM2AP), and hydrolyze...
Autores principales: | Tropak, Michael B, Yonekawa, Sayuri, Karumuthil-Melethil, Subha, Thompson, Patrick, Wakarchuk, Warren, Gray, Steven J, Walia, Jagdeep S, Mark, Brian L, Mahuran, Don |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4774620/ https://www.ncbi.nlm.nih.gov/pubmed/26966698 http://dx.doi.org/10.1038/mtm.2015.57 |
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