1kws: Difference between revisions

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~~[[Image:1kws.png|left|200px]]~~

===CRYSTAL STRUCTURE OF BETA1,3-GLUCURONYLTRANSFERASE I IN COMPLEX WITH THE ACTIVE UDP-GLCUA DONOR===

===~~CRYSTAL STRUCTURE OF BETA1~~,~~3-GLUCURONYLTRANSFERASE I IN COMPLEX WITH THE ACTIVE UDP-GLCUA DONOR===~~

==Disease==

[[http://www.uniprot.org/uniprot/B3GA3_HUMAN B3GA3_HUMAN]] Defects in B3GAT3 are the cause of multiple joint dislocations short stature craniofacial dysmorphism and congenital heart defects (JDSSDHD) [MIM:[http://omim.org/entry/245600 245600]]. An autosomal recessive disease characterized by dysmorphic facies, bilateral dislocations of the elbows, hips, and knees, clubfeet, and short stature, as well as cardiovascular defects.<ref>PMID:21763480</ref>

~~{{ABSTRACT_PUBMED_11950836}}~~

==Function==

[[http://www.uniprot.org/uniprot/B3GA3_HUMAN B3GA3_HUMAN]] Glycosaminoglycans biosynthesis. Involved in forming the linkage tetrasaccharide present in heparan sulfate and chondroitin sulfate. Transfers a glucuronic acid moiety from the uridine diphosphate-glucuronic acid (UDP-GlcUA) to the common linkage region trisaccharide Gal-beta-1,3-Gal-beta-1,4-Xyl covalently bound to a Ser residue at the glycosaminylglycan attachment site of proteoglycans. Can also play a role in the biosynthesis of l2/HNK-1 carbohydrate epitope on glycoproteins. Shows strict specificity for Gal-beta-1,3-Gal-beta-1,4-Xyl, exhibiting negligible incorporation into other galactoside substrates including Galbeta1-3Gal beta1-O-benzyl, Galbeta1-4GlcNAc and Galbeta1-4Glc.

==About this Structure==

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==Reference==

[[Category: Homo sapiens]]

[[Category: Darden, T A.]]

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@@ Line 1: / Line 1: @@
-[[Image:1kws.png|left|200px]]
 {{STRUCTURE_1kws|  PDB=1kws  |  SCENE=  }}
+===CRYSTAL STRUCTURE OF BETA1,3-GLUCURONYLTRANSFERASE I IN COMPLEX WITH THE ACTIVE UDP-GLCUA DONOR===
+{{ABSTRACT_PUBMED_11950836}}
-===CRYSTAL STRUCTURE OF BETA1,3-GLUCURONYLTRANSFERASE I IN COMPLEX WITH THE ACTIVE UDP-GLCUA DONOR===
+==Disease==
+[[http://www.uniprot.org/uniprot/B3GA3_HUMAN B3GA3_HUMAN]] Defects in B3GAT3 are the cause of multiple joint dislocations short stature craniofacial dysmorphism and congenital heart defects (JDSSDHD) [MIM:[http://omim.org/entry/245600 245600]]. An autosomal recessive disease characterized by dysmorphic facies, bilateral dislocations of the elbows, hips, and knees, clubfeet, and short stature, as well as cardiovascular defects.<ref>PMID:21763480</ref>
-{{ABSTRACT_PUBMED_11950836}}
+==Function==
+[[http://www.uniprot.org/uniprot/B3GA3_HUMAN B3GA3_HUMAN]] Glycosaminoglycans biosynthesis. Involved in forming the linkage tetrasaccharide present in heparan sulfate and chondroitin sulfate. Transfers a glucuronic acid moiety from the uridine diphosphate-glucuronic acid (UDP-GlcUA) to the common linkage region trisaccharide Gal-beta-1,3-Gal-beta-1,4-Xyl covalently bound to a Ser residue at the glycosaminylglycan attachment site of proteoglycans. Can also play a role in the biosynthesis of l2/HNK-1 carbohydrate epitope on glycoproteins. Shows strict specificity for Gal-beta-1,3-Gal-beta-1,4-Xyl, exhibiting negligible incorporation into other galactoside substrates including Galbeta1-3Gal beta1-O-benzyl, Galbeta1-4GlcNAc and Galbeta1-4Glc.
 ==About this Structure==
@@ Line 11: / Line 13: @@
 ==Reference==
-<ref group="xtra">PMID:011950836</ref><references group="xtra"/>
+<ref group="xtra">PMID:011950836</ref><references group="xtra"/><references/>
 [[Category: Homo sapiens]]
 [[Category: Darden, T A.]]