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| Content Provider | PubMed Central |
|---|---|
| Author | Mark, Brian L. Mahuran, Don J. Cherney, Maia M. Zhao, Dalian Knapp, Spencer James, Michael N. G. |
| Abstract | In humans, two major β-hexosaminidase isoenzymes exist: Hex A and Hex B. Hex A is a heterodimer of subunits α and β (60% identity), whereas Hex B is a homodimer of β-subunits. Interest in human β-hexosaminidase stems from its association with Tay–Sachs and Sandhoff disease; these are prototypical lysosomal storage disorders resulting from the abnormal accumulation of GM2-ganglioside (GM2). Hex A degrades GM2 by removing a terminal N-acetyl-D-galactosamine (β-GalNAc) residue, and this activity requires the GM2–activator, a protein which solubilizes the ganglioside for presentation to Hex A. We present here the crystal structure of human Hex B, alone (2.4 Å) and in complex with the mechanistic inhibitors GalNAc-isofagomine (2.2 Å) or NAG-thiazoline (2.5 Å). From these, and the known X-ray structure of the GM2–activator, we have modeled Hex A in complex with the activator and ganglioside. Together, our crystallographic and modeling data demonstrate how α and β-subunits dimerize to form either Hex A or Hex B, how these isoenzymes hydrolyze diverse substrates, and how many documented point mutations cause Sandhoff disease (β-subunit mutations) and Tay–Sachs disease (α-subunit mutations). |
| Starting Page | 1093 |
| File Format | |
| ISSN | 10898638 |
| e-ISSN | 10898638 |
| Journal | Journal of molecular biology |
| Issue Number | 5 |
| Volume Number | 327 |
| Language | English |
| Publisher Date | 2003-04-11 |
| Access Restriction | Open |
| Subject Keyword | Research in Higher Education |
| Content Type | Text |
| Resource Type | Article |
| Subject | Structural Biology Molecular Biology Biophysics |
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