2o4h: Difference between revisions

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~~[[Image:2o4h.png|left|200px]]~~

===Human brain aspartoacylase complex with intermediate analog (N-phosphonomethyl-L-aspartate)===

===~~Human brain aspartoacylase complex with intermediate analog~~ (~~N-phosphonomethyl-L-aspartate~~)~~===~~

==Disease==

[[http://www.uniprot.org/uniprot/ACY2_HUMAN ACY2_HUMAN]] Defects in ASPA are the cause of Canavan disease (CAND) [MIM:[http://omim.org/entry/271900 271900]]; also known as spongy degeneration of the brain. CAND is a rare neurodegenerative condition of infancy or childhood characterized by white matter vacuolization and demeylination that gives rise to a spongy appearance. The clinical features are onset in early infancy, atonia of neck muscles, hypotonia, hyperextension of legs and flexion of arms, blindness, severe mental defect, megalocephaly, and death by 18 months on the average.<ref>PMID:8252036</ref><ref>PMID:12706335</ref><ref>PMID:8023850</ref><ref>PMID:7668285</ref><ref>PMID:7599639</ref><ref>PMID:8659549</ref><ref>PMID:9452117</ref><ref>PMID:10564886</ref><ref>PMID:10407784</ref><ref>PMID:10909858</ref><ref>PMID:12638939</ref><ref>PMID:12205125</ref>

~~{{ABSTRACT_PUBMED_18293939}}~~

==Function==

[[http://www.uniprot.org/uniprot/ACY2_HUMAN ACY2_HUMAN]] Catalyzes the deacetylation of N-acetylaspartic acid (NAA) to produce acetate and L-aspartate. NAA occurs in high concentration in brain and its hydrolysis NAA plays a significant part in the maintenance of intact white matter. In other tissues it act as a scavenger of NAA from body fluids.

==About this Structure==

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==Reference==

[[Category: Aspartoacylase]]

[[Category: Homo sapiens]]

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@@ Line 1: / Line 1: @@
-[[Image:2o4h.png|left|200px]]
 {{STRUCTURE_2o4h|  PDB=2o4h  |  SCENE=  }}
+===Human brain aspartoacylase complex with intermediate analog (N-phosphonomethyl-L-aspartate)===
+{{ABSTRACT_PUBMED_18293939}}
-===Human brain aspartoacylase complex with intermediate analog (N-phosphonomethyl-L-aspartate)===
+==Disease==
+[[http://www.uniprot.org/uniprot/ACY2_HUMAN ACY2_HUMAN]] Defects in ASPA are the cause of Canavan disease (CAND) [MIM:[http://omim.org/entry/271900 271900]]; also known as spongy degeneration of the brain. CAND is a rare neurodegenerative condition of infancy or childhood characterized by white matter vacuolization and demeylination that gives rise to a spongy appearance. The clinical features are onset in early infancy, atonia of neck muscles, hypotonia, hyperextension of legs and flexion of arms, blindness, severe mental defect, megalocephaly, and death by 18 months on the average.<ref>PMID:8252036</ref><ref>PMID:12706335</ref><ref>PMID:8023850</ref><ref>PMID:7668285</ref><ref>PMID:7599639</ref><ref>PMID:8659549</ref><ref>PMID:9452117</ref><ref>PMID:10564886</ref><ref>PMID:10407784</ref><ref>PMID:10909858</ref><ref>PMID:12638939</ref><ref>PMID:12205125</ref>
-{{ABSTRACT_PUBMED_18293939}}
+==Function==
+[[http://www.uniprot.org/uniprot/ACY2_HUMAN ACY2_HUMAN]] Catalyzes the deacetylation of N-acetylaspartic acid (NAA) to produce acetate and L-aspartate. NAA occurs in high concentration in brain and its hydrolysis NAA plays a significant part in the maintenance of intact white matter. In other tissues it act as a scavenger of NAA from body fluids.
 ==About this Structure==
@@ Line 14: / Line 16: @@
 ==Reference==
-<ref group="xtra">PMID:018293939</ref><references group="xtra"/>
+<ref group="xtra">PMID:018293939</ref><references group="xtra"/><references/>
 [[Category: Aspartoacylase]]
 [[Category: Homo sapiens]]