Saposin: Difference between revisions
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{{STRUCTURE_2dob| PDB=2dob | SIZE=400| SCENE= |right|CAPTION=Human saposin A complex with Ca+2 ion, [[2dob]] }} | {{STRUCTURE_2dob| PDB=2dob | SIZE=400| SCENE= |right|CAPTION=Human saposin A complex with Ca+2 ion, [[2dob]] }} | ||
== Function == | |||
'''Saposin''' (Sap) is a small protein which functions as activator of lipid-degrading enzymes. They act by isolating the lipid substrate from the membrane. Sap is synthesized as a precursor – prosaposin – which contain 4 SapB active domains and 2 SapA domains which are cleaved off<ref>PMID:2001789</ref>. | |||
'''Saposin''' | *'''Saposin A and C''' stimulate hydrolysis of methylumbelliferyl β-galactoside by β-glucosylceramidase and of galactocerebrocide by β-galactosylceramidase<ref>PMID:2717620</ref>. For more details see [[Molecular Playground/Saposin C]].<br /> | ||
*'''Saposin B''' facilitates lipid binding to CD1d<ref>PMID:17372201</ref>.<br /> | |||
*'''Saposin D''' stimulates acid ceramidase activity<ref>PMID:8203897</ref>.<br /> | |||
== Disease == | |||
Mutations in saposin B are autosomal recessive trait resulting in clinical metachromatic leukodystrophy<ref>PMID:17616409</ref>. Mutations in saposin D cause urinary system defects<ref>PMID:15345707</ref>. | |||
== 3D Structures of Saposin == | == 3D Structures of Saposin == | ||
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**[[3bqp]], [[3bqq]], [[2r1q]], [[2rb3]] - hSapD residues 405-484<br /> | **[[3bqp]], [[3bqq]], [[2r1q]], [[2rb3]] - hSapD residues 405-484<br /> | ||
}} | }} | ||
== References == | |||
<references/> | |||
[[Category:Topic Page]] | [[Category:Topic Page]] | ||
