1n69
From Proteopedia
(New page: 200px<br /> <applet load="1n69" size="450" color="white" frame="true" align="right" spinBox="true" caption="1n69, resolution 2.20Å" /> '''Crystal structure o...) |
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| - | [[Image:1n69.gif|left|200px]]<br /> | + | [[Image:1n69.gif|left|200px]]<br /><applet load="1n69" size="350" color="white" frame="true" align="right" spinBox="true" |
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caption="1n69, resolution 2.20Å" /> | caption="1n69, resolution 2.20Å" /> | ||
'''Crystal structure of human saposin B'''<br /> | '''Crystal structure of human saposin B'''<br /> | ||
==Overview== | ==Overview== | ||
| - | Saposin B is a small, nonenzymatic glycosphingolipid activator protein | + | Saposin B is a small, nonenzymatic glycosphingolipid activator protein required for the breakdown of cerebroside sulfates (sulfatides) within the lysosome. The protein can extract target lipids from membranes, forming soluble protein-lipid complexes that are recognized by arylsulfatase A. The crystal structure of human saposin B reveals an unusual shell-like dimer consisting of a monolayer of alpha-helices enclosing a large hydrophobic cavity. Although the secondary structure of saposin B is similar to that of the known monomeric members of the saposin-like superfamily, the helices are repacked into a different tertiary arrangement to form the homodimer. A comparison of the two forms of the saposin B dimer suggests that extraction of target lipids from membranes involves a conformational change that facilitates access to the inner cavity. |
==Disease== | ==Disease== | ||
| - | Known diseases associated with this structure: Combined SAP deficiency OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=176801 176801]], Gaucher disease, atypical OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=176801 176801]], Metachromatic leukodystrophy due to | + | Known diseases associated with this structure: Combined SAP deficiency OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=176801 176801]], Gaucher disease, atypical OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=176801 176801]], Krabbe disease, atypical OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=176801 176801]], Metachromatic leukodystrophy due to SAP-b deficiency OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=176801 176801]] |
==About this Structure== | ==About this Structure== | ||
| - | 1N69 is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with PEH as [http://en.wikipedia.org/wiki/ligand ligand]. Full crystallographic information is available from [http:// | + | 1N69 is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with <scene name='pdbligand=PEH:'>PEH</scene> as [http://en.wikipedia.org/wiki/ligand ligand]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1N69 OCA]. |
==Reference== | ==Reference== | ||
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[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Single protein]] | [[Category: Single protein]] | ||
| - | [[Category: Ahn, V | + | [[Category: Ahn, V E.]] |
| - | [[Category: Faull, K | + | [[Category: Faull, K F.]] |
| - | [[Category: Fluharty, A | + | [[Category: Fluharty, A L.]] |
| - | [[Category: Prive, G | + | [[Category: Prive, G G.]] |
| - | [[Category: Whitelegge, J | + | [[Category: Whitelegge, J P.]] |
[[Category: PEH]] | [[Category: PEH]] | ||
[[Category: glycosphingolipid activator protein]] | [[Category: glycosphingolipid activator protein]] | ||
[[Category: lipid binding protein]] | [[Category: lipid binding protein]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 14:02:41 2008'' |
Revision as of 12:02, 21 February 2008
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Crystal structure of human saposin B
Contents |
Overview
Saposin B is a small, nonenzymatic glycosphingolipid activator protein required for the breakdown of cerebroside sulfates (sulfatides) within the lysosome. The protein can extract target lipids from membranes, forming soluble protein-lipid complexes that are recognized by arylsulfatase A. The crystal structure of human saposin B reveals an unusual shell-like dimer consisting of a monolayer of alpha-helices enclosing a large hydrophobic cavity. Although the secondary structure of saposin B is similar to that of the known monomeric members of the saposin-like superfamily, the helices are repacked into a different tertiary arrangement to form the homodimer. A comparison of the two forms of the saposin B dimer suggests that extraction of target lipids from membranes involves a conformational change that facilitates access to the inner cavity.
Disease
Known diseases associated with this structure: Combined SAP deficiency OMIM:[176801], Gaucher disease, atypical OMIM:[176801], Krabbe disease, atypical OMIM:[176801], Metachromatic leukodystrophy due to SAP-b deficiency OMIM:[176801]
About this Structure
1N69 is a Single protein structure of sequence from Homo sapiens with as ligand. Full crystallographic information is available from OCA.
Reference
Crystal structure of saposin B reveals a dimeric shell for lipid binding., Ahn VE, Faull KF, Whitelegge JP, Fluharty AL, Prive GG, Proc Natl Acad Sci U S A. 2003 Jan 7;100(1):38-43. Epub 2002 Dec 23. PMID:12518053
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