4ee5
From Proteopedia
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{{STRUCTURE_4ee5| PDB=4ee5 | SCENE= }} | {{STRUCTURE_4ee5| PDB=4ee5 | SCENE= }} | ||
===Crystal structure of human M340H-beta-1,4-galactosyltransferase-1 (M340H-B4GAL-T1) in complex with trisaccharide from Lacto-N-neotetraose=== | ===Crystal structure of human M340H-beta-1,4-galactosyltransferase-1 (M340H-B4GAL-T1) in complex with trisaccharide from Lacto-N-neotetraose=== | ||
- | {{ | + | {{ABSTRACT_PUBMED_22740701}} |
==Disease== | ==Disease== | ||
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==About this Structure== | ==About this Structure== | ||
- | [[4ee5]] is a 3 chain structure with sequence from [http://en.wikipedia.org/wiki/ | + | [[4ee5]] is a 3 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4EE5 OCA]. |
==See Also== | ==See Also== | ||
- | *[[ | + | *[[Glycosyltransferase|Glycosyltransferase]] |
- | [[Category: | + | |
+ | ==Reference== | ||
+ | <ref group="xtra">PMID:022740701</ref><references group="xtra"/><references/> | ||
+ | [[Category: Human]] | ||
[[Category: Qasba, P K.]] | [[Category: Qasba, P K.]] | ||
[[Category: Ramakrishnan, B.]] | [[Category: Ramakrishnan, B.]] |
Revision as of 06:41, 26 March 2014
Contents |
Crystal structure of human M340H-beta-1,4-galactosyltransferase-1 (M340H-B4GAL-T1) in complex with trisaccharide from Lacto-N-neotetraose
Template:ABSTRACT PUBMED 22740701
Disease
[B4GT1_HUMAN] Defects in B4GALT1 are the cause of congenital disorder of glycosylation type 2D (CDG2D) [MIM:607091]. CDGs are a family of severe inherited diseases caused by a defect in protein N-glycosylation. They are characterized by under-glycosylated serum proteins. These multisystem disorders present with a wide variety of clinical features, such as disorders of the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions.
Function
[B4GT1_HUMAN] The Golgi complex form catalyzes the production of lactose in the lactating mammary gland and could also be responsible for the synthesis of complex-type N-linked oligosaccharides in many glycoproteins as well as the carbohydrate moieties of glycolipids. The cell surface form functions as a recognition molecule during a variety of cell to cell and cell to matrix interactions, as those occurring during development and egg fertilization, by binding to specific oligosaccharide ligands on opposing cells or in the extracellular matrix.
About this Structure
4ee5 is a 3 chain structure with sequence from Human. Full crystallographic information is available from OCA.
See Also
Reference
- Ramakrishnan B, Boeggeman E, Qasba PK. Binding of N-acetylglucosamine (GlcNAc) beta1-6-branched oligosaccharide acceptors to beta4-galactosyltransferase I reveals a new ligand binding mode. J Biol Chem. 2012 Aug 17;287(34):28666-74. doi: 10.1074/jbc.M112.373514. Epub, 2012 Jun 27. PMID:22740701 doi:http://dx.doi.org/10.1074/jbc.M112.373514