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| [[Image:1g13.gif|left|200px]] | | {{Seed}} |
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| {{STRUCTURE_1g13| PDB=1g13 | SCENE= }} | | {{STRUCTURE_1g13| PDB=1g13 | SCENE= }} |
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| '''HUMAN GM2 ACTIVATOR STRUCTURE'''
| | ===HUMAN GM2 ACTIVATOR STRUCTURE=== |
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| ==Overview==
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| GM2 activator protein (GM2-AP) belongs to a small group of non- enzymatic lysosomal proteins that act as cofactors in the sequential degradation of gangliosides. It has been postulated that GM2-AP extracts single GM2 molecules from membranes and presents them in soluble form to beta-hexosaminidase A for cleavage of N-acetyl-d-galactosamine and conversion to GM3. The high affinity of GM2-AP for GM2 is based on specfic recognition of the oligosaccharide moiety as well as the ceramide lipid tail. Genetic defects in GM2-AP result in an atypical form of Tay-Sachs disease known as variant AB GM2 gangliosidosis. The 2.0 A resolution crystal structure of GM2-AP reported here reveals a previously unobserved fold whose main feature is an eight-stranded cup-shaped anti-parallel beta-pleated sheet. The striking feature of the GM2-AP structure is that it possesses an accessible central hydrophobic cavity rather than a buried hydrophobic core. The dimensions of this cavity (12 Ax14 Ax22 A) are suitable for binding 18-carbon lipid acyl chains. Flexible surface loops and a short alpha-helix decorate the mouth of the beta-cup and may control lipid entry to the cavity.
| | The line below this paragraph, {{ABSTRACT_PUBMED_11090283}}, adds the Publication Abstract to the page |
| | (as it appears on PubMed at http://www.pubmed.gov), where 11090283 is the PubMed ID number. |
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| | {{ABSTRACT_PUBMED_11090283}} |
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| ==About this Structure== | | ==About this Structure== |
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| [[Category: Wright, C S.]] | | [[Category: Wright, C S.]] |
| [[Category: Beta cup]] | | [[Category: Beta cup]] |
| ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May 2 16:59:59 2008'' | | |
| | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 1 04:14:44 2008'' |
