3lpp: Difference between revisions
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{{STRUCTURE_3lpp| PDB=3lpp | SCENE= }} | {{STRUCTURE_3lpp| PDB=3lpp | SCENE= }} | ||
===Crystal complex of N-terminal sucrase-isomaltase with kotalanol=== | ===Crystal complex of N-terminal sucrase-isomaltase with kotalanol=== | ||
{{ABSTRACT_PUBMED_20356844}} | {{ABSTRACT_PUBMED_20356844}} | ||
==Disease== | ==Disease== | ||
[[http://www.uniprot.org/uniprot/SUIS_HUMAN SUIS_HUMAN]] Congenital sucrase-isomaltase deficiency. Defects in SI are the cause of congenital sucrase-isomaltase deficiency (CSID) [MIM:[http://omim.org/entry/222900 222900]]; also known as disaccharide intolerance I. CSID is an autosomal recessive intestinal disorder that is clinically characterized by fermentative diarrhea, abdominal pain, and cramps upon ingestion of sugar. The symptoms are the consequence of absent or drastically reduced enzymatic activities of sucrase and isomaltase. The prevalence of CSID is 0.02 % in individuals of European descent and appears to be much higher in Greenland, Alaskan, and Canadian native people. CSID arises due to post-translational perturbations in the intracellular transport, polarized sorting, aberrant processing, and defective function of SI.<ref>PMID:8609217</ref> <ref>PMID:10903344</ref> <ref>PMID:11340066</ref> <ref>PMID:14724820</ref> <ref>PMID:16329100</ref> | |||
==Function== | |||
[[http://www.uniprot.org/uniprot/SUIS_HUMAN SUIS_HUMAN]] Plays an important role in the final stage of carbohydrate digestion. Isomaltase activity is specific for both alpha-1,4- and alpha-1,6-oligosaccharides.<ref>PMID:20356844</ref> | |||
==About this Structure== | ==About this Structure== | ||
[[3lpp]] is a 4 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3LPP OCA]. | |||
==Reference== | ==Reference== | ||
<ref group="xtra">PMID: | <ref group="xtra">PMID:020356844</ref><references group="xtra"/><references/> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Oligo-1,6-glucosidase]] | [[Category: Oligo-1,6-glucosidase]] | ||
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[[Category: Membrane]] | [[Category: Membrane]] | ||
[[Category: Multifunctional enzyme]] | [[Category: Multifunctional enzyme]] | ||
[[Category: Signal-anchor]] | [[Category: Signal-anchor]] | ||
[[Category: Sulfation]] | [[Category: Sulfation]] | ||
[[Category: Transmembrane]] | [[Category: Transmembrane]] | ||