Complete localization of disulfide bonds in GM2 activator protein

Schütte, C. G.; Lemm, T.; Sandhoff, K.; Glombitza, Gereon

doi:10.1002/pro.5560070421

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Complete localization of disulfide bonds in GM2 activator protein

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Glombitza, Gereon
Department of Bioorganic Chemistry, Prof. Dr. W. Boland, MPI for Chemical Ecology, Max Planck Society;

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http://dx.doi.org/10.1002/pro.5560070421
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引用

Schütte, C. G., Lemm, T., Sandhoff, K., & Glombitza, G. (1998). Complete localization of disulfide bonds in GM2 activator protein. Protein Science, 7(4), 1039-1045. doi:10.1002/pro.5560070421.

引用: https://hdl.handle.net/11858/00-001M-0000-0012-A364-B

要旨

Lysosomal degradation of ganglioside GM2 by hexosaminidase A requires the presence of a small, non-enzymatic cofactor, the GM2-activator protein (GM2AP). Lack of functional protein leads to the AB variant of GM2-gangliosidosis, a fatal lysosomal storage