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...

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Bibliografske podrobnosti
izdano v:Mol Ther Methods Clin Dev
Main Authors: Tropak, Michael B, Yonekawa, Sayuri, Karumuthil-Melethil, Subha, Thompson, Patrick, Wakarchuk, Warren, Gray, Steven J, Walia, Jagdeep S, Mark, Brian L, Mahuran, Don
Format: Artigo
Jezik:Inglês
Izdano: Nature Publishing Group 2016
Teme:
Article
Online dostop:https://ncbi.nlm.nih.gov/pmc/articles/PMC4774620/
https://ncbi.nlm.nih.gov/pubmed/26966698
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/mtm.2015.57
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