2beu: Difference between revisions

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

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===REACTIVITY MODULATION OF HUMAN BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE BY AN INTERNAL MOLECULAR SWITCH===

==Disease==

[[http://www.uniprot.org/uniprot/ODBA_HUMAN ODBA_HUMAN]] Defects in BCKDHA are a cause of maple syrup urine disease type IA (MSUD1A) [MIM:[http://omim.org/entry/248600 248600]]. MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine.<ref>PMID:2060625</ref><ref>PMID:8037208</ref><ref>PMID:2703538</ref><ref>PMID:2241958</ref><ref>PMID:1867199</ref><ref>PMID:1885764</ref><ref>PMID:8161368</ref><ref>PMID:7883996</ref> [[http://www.uniprot.org/uniprot/ODBB_HUMAN ODBB_HUMAN]] Defects in BCKDHB are the cause of maple syrup urine disease type IB (MSUD1B) [MIM:[http://omim.org/entry/248600 248600]]. MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine.<ref>PMID:8161368</ref><ref>PMID:11509994</ref><ref>PMID:22326532</ref>

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==Function==

~~The line below this paragraph, {{ABSTRACT_PUBMED_16472748}}, adds~~ the ~~Publication Abstract~~ to ~~the page~~

[[http://www.uniprot.org/uniprot/ODBA_HUMAN ODBA_HUMAN]] The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). [[http://www.uniprot.org/uniprot/ODBB_HUMAN ODBB_HUMAN]] The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).

(~~as it appears on PubMed at~~ http://www.~~pubmed~~.~~gov~~)~~, where 16472748 is the PubMed ID number~~.

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~~{{ABSTRACT_PUBMED_16472748}}~~

==About this Structure==

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==Reference==

[[Category: Homo sapiens]]

[[Category: Brautigam, C A.]]

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@@ Line 1: / Line 1: @@
-[[Image:2beu.png|left|200px]]
-<!--
-The line below this paragraph, containing "STRUCTURE_2beu", creates the "Structure Box" on the page.
-You may change the PDB parameter (which sets the PDB file loaded into the applet)
-or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
-or leave the SCENE parameter empty for the default display.
--->
 {{STRUCTURE_2beu|  PDB=2beu  |  SCENE=  }}
 ===REACTIVITY MODULATION OF HUMAN BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE BY AN INTERNAL MOLECULAR SWITCH===
+{{ABSTRACT_PUBMED_16472748}}
+==Disease==
+[[http://www.uniprot.org/uniprot/ODBA_HUMAN ODBA_HUMAN]] Defects in BCKDHA are a cause of maple syrup urine disease type IA (MSUD1A) [MIM:[http://omim.org/entry/248600 248600]]. MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine.<ref>PMID:2060625</ref><ref>PMID:8037208</ref><ref>PMID:2703538</ref><ref>PMID:2241958</ref><ref>PMID:1867199</ref><ref>PMID:1885764</ref><ref>PMID:8161368</ref><ref>PMID:7883996</ref> [[http://www.uniprot.org/uniprot/ODBB_HUMAN ODBB_HUMAN]] Defects in BCKDHB are the cause of maple syrup urine disease type IB (MSUD1B) [MIM:[http://omim.org/entry/248600 248600]]. MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine.<ref>PMID:8161368</ref><ref>PMID:11509994</ref><ref>PMID:22326532</ref>
-<!--
+==Function==
-The line below this paragraph, {{ABSTRACT_PUBMED_16472748}}, adds the Publication Abstract to the page
+[[http://www.uniprot.org/uniprot/ODBA_HUMAN ODBA_HUMAN]] The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). [[http://www.uniprot.org/uniprot/ODBB_HUMAN ODBB_HUMAN]] The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
-(as it appears on PubMed at http://www.pubmed.gov), where 16472748 is the PubMed ID number.
--->
-{{ABSTRACT_PUBMED_16472748}}
 ==About this Structure==
@@ Line 22: / Line 13: @@
 ==Reference==
-<ref group="xtra">PMID:016472748</ref><ref group="xtra">PMID:015166214</ref><ref group="xtra">PMID:012902323</ref><ref group="xtra">PMID:011069910</ref><references group="xtra"/>
+<ref group="xtra">PMID:016472748</ref><ref group="xtra">PMID:015166214</ref><ref group="xtra">PMID:012902323</ref><ref group="xtra">PMID:011069910</ref><references group="xtra"/><references/>
 [[Category: Homo sapiens]]
 [[Category: Brautigam, C A.]]