2zqq: Difference between revisions

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~~[[Image:2zqq.png|left|200px]]~~

===Crystal structure of human AUH (3-methylglutaconyl-coa hydratase) mixed with (AUUU)24A RNA===

===~~Crystal structure~~ of ~~human AUH~~ (3-~~methylglutaconyl~~-~~coa hydratase) mixed with~~ (~~AUUU~~)~~24A RNA===~~

==Disease==

[[http://www.uniprot.org/uniprot/AUHM_HUMAN AUHM_HUMAN]] Defects in AUH are the cause of 3-methylglutaconic aciduria type 1 (MGA1) [MIM:[http://omim.org/entry/250950 250950]]. MGA1 is an inborn error of leucine metabolism. It leads to an autosomal recessive syndrome with variable clinical phenotype, ranging from delayed speech development to severe psychomotor retardation, coma, failure to thrive, metabolic acidosis and dystonia. MGA1 can be distinguished from other forms of MGA by the pattern of metabolite excretion: 3-methylglutaconic acid levels are higher than those detected in other forms, whereas methylglutaric acid levels are usually only slightly elevated, and there is a high level of 3-hydroxyisovaleric acid excretion (not present in other MGA forms).<ref>PMID:12434311</ref><ref>PMID:12655555</ref>

~~{{ABSTRACT_PUBMED_18831052}}~~

==Function==

[[http://www.uniprot.org/uniprot/AUHM_HUMAN AUHM_HUMAN]] Catalyzes the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs.<ref>PMID:7892223</ref><ref>PMID:12434311</ref><ref>PMID:11738050</ref><ref>PMID:12655555</ref>

==About this Structure==

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==Reference==

[[Category: Homo sapiens]]

[[Category: Methylglutaconyl-CoA hydratase]]

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@@ Line 1: / Line 1: @@
-[[Image:2zqq.png|left|200px]]
 {{STRUCTURE_2zqq|  PDB=2zqq  |  SCENE=  }}
+===Crystal structure of human AUH (3-methylglutaconyl-coa hydratase) mixed with (AUUU)24A RNA===
+{{ABSTRACT_PUBMED_18831052}}
-===Crystal structure of human AUH (3-methylglutaconyl-coa hydratase) mixed with (AUUU)24A RNA===
+==Disease==
+[[http://www.uniprot.org/uniprot/AUHM_HUMAN AUHM_HUMAN]] Defects in AUH are the cause of 3-methylglutaconic aciduria type 1 (MGA1) [MIM:[http://omim.org/entry/250950 250950]]. MGA1 is an inborn error of leucine metabolism. It leads to an autosomal recessive syndrome with variable clinical phenotype, ranging from delayed speech development to severe psychomotor retardation, coma, failure to thrive, metabolic acidosis and dystonia. MGA1 can be distinguished from other forms of MGA by the pattern of metabolite excretion: 3-methylglutaconic acid levels are higher than those detected in other forms, whereas methylglutaric acid levels are usually only slightly elevated, and there is a high level of 3-hydroxyisovaleric acid excretion (not present in other MGA forms).<ref>PMID:12434311</ref><ref>PMID:12655555</ref>
-{{ABSTRACT_PUBMED_18831052}}
+==Function==
+[[http://www.uniprot.org/uniprot/AUHM_HUMAN AUHM_HUMAN]] Catalyzes the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs.<ref>PMID:7892223</ref><ref>PMID:12434311</ref><ref>PMID:11738050</ref><ref>PMID:12655555</ref>
 ==About this Structure==
@@ Line 14: / Line 16: @@
 ==Reference==
-<ref group="xtra">PMID:018831052</ref><references group="xtra"/>
+<ref group="xtra">PMID:018831052</ref><references group="xtra"/><references/>
 [[Category: Homo sapiens]]
 [[Category: Methylglutaconyl-CoA hydratase]]