3bqq

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Template:STRUCTURE 3bqq

Crystal Structure of Human Saposin D (triclinic)


Contents

Overview

Saposin D is a sphingolipid activator protein required for the lysosomal breakdown of ceramide to a fatty acid and sphingosine by acid ceramidase. The crystal structure of saposin D has been determined in two different crystal forms, resulting in a total of six crystallographically independent views of this small 80-amino-acid protein. All of the structures are highly similar and reveal the monomeric form of the saposin fold previously seen in the crystal structures of saposins A and C. Saposin D is slightly more compact than the related saposins A and C owing to a slight repositioning of the ;stem' and ;hairpin' regions of the protein.

Disease

Known disease 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

3BQQ is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Structures of the human ceramide activator protein saposin D., Popovic K, Prive GG, Acta Crystallogr D Biol Crystallogr. 2008 May;64(Pt 5):589-94. Epub 2008, Apr 19. PMID:18453694 Page seeded by OCA on Thu May 22 22:35:30 2008

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