1eyb: Difference between revisions

Line 4:

==Overview==

Homogentisate dioxygenase (HGO) cleaves the aromatic ring during the, metabolic degradation of Phe and Tyr. HGO deficiency causes alkaptonuria, (AKU), the first human disease shown to be inherited as a recessive, Mendelian trait. Crystal structures of apo-HGO and HGO containing an iron, ion have been determined at 1.9 and 2.3 A resolution, respectively. The, HGO protomer, which contains a 280-residue N-terminal domain and a, 140-residue C-terminal domain, associates as a hexamer arranged as a dimer, of trimers. The active site iron ion is coordinated near the interface, between subunits in the HGO trimer by a Glu and two His side chains. HGO, represents a new structural class of dioxygenases. The largest group of, AKU associated missense mutations affect residues located in regions of, contact between subunits.

Homogentisate dioxygenase (HGO) cleaves the aromatic ring during the metabolic degradation of Phe and Tyr. HGO deficiency causes alkaptonuria (AKU), the first human disease shown to be inherited as a recessive Mendelian trait. Crystal structures of apo-HGO and HGO containing an iron ion have been determined at 1.9 and 2.3 A resolution, respectively. The HGO protomer, which contains a 280-residue N-terminal domain and a 140-residue C-terminal domain, associates as a hexamer arranged as a dimer of trimers. The active site iron ion is coordinated near the interface between subunits in the HGO trimer by a Glu and two His side chains. HGO represents a new structural class of dioxygenases. The largest group of AKU associated missense mutations affect residues located in regions of contact between subunits.

==Disease==

Line 17:

[[Category: Homogentisate 1,2-dioxygenase]]

[[Category: Single protein]]

[[Category: Cordoba, S.M.de.]]

[[Category: Cordoba, S M.de.]]

[[Category: Mueller, H.A.]]

[[Category: Mueller, H A.]]

[[Category: Penalva, M.A.]]

[[Category: Penalva, M A.]]

[[Category: Timm, D.E.]]

[[Category: Timm, D E.]]

[[Category: Titus, G.P.]]

[[Category: Titus, G P.]]

[[Category: beta sandwich]]

[[Category: jelly roll]]

''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on ~~Fri~~ Feb ~~15 15~~:45:13 2008''

''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 12:32:49 2008''

Navigation menu

Page actions

Page actions

Personal tools

Navigation

Search

Tools

@@ Line 4: / Line 4: @@
 ==Overview==
-Homogentisate dioxygenase (HGO) cleaves the aromatic ring during the, metabolic degradation of Phe and Tyr. HGO deficiency causes alkaptonuria, (AKU), the first human disease shown to be inherited as a recessive, Mendelian trait. Crystal structures of apo-HGO and HGO containing an iron, ion have been determined at 1.9 and 2.3 A resolution, respectively. The, HGO protomer, which contains a 280-residue N-terminal domain and a, 140-residue C-terminal domain, associates as a hexamer arranged as a dimer, of trimers. The active site iron ion is coordinated near the interface, between subunits in the HGO trimer by a Glu and two His side chains. HGO, represents a new structural class of dioxygenases. The largest group of, AKU associated missense mutations affect residues located in regions of, contact between subunits.
+Homogentisate dioxygenase (HGO) cleaves the aromatic ring during the metabolic degradation of Phe and Tyr. HGO deficiency causes alkaptonuria (AKU), the first human disease shown to be inherited as a recessive Mendelian trait. Crystal structures of apo-HGO and HGO containing an iron ion have been determined at 1.9 and 2.3 A resolution, respectively. The HGO protomer, which contains a 280-residue N-terminal domain and a 140-residue C-terminal domain, associates as a hexamer arranged as a dimer of trimers. The active site iron ion is coordinated near the interface between subunits in the HGO trimer by a Glu and two His side chains. HGO represents a new structural class of dioxygenases. The largest group of AKU associated missense mutations affect residues located in regions of contact between subunits.
 ==Disease==
@@ Line 17: / Line 17: @@
 [[Category: Homogentisate 1,2-dioxygenase]]
 [[Category: Single protein]]
-[[Category: Cordoba, S.M.de.]]
+[[Category: Cordoba, S M.de.]]
-[[Category: Mueller, H.A.]]
+[[Category: Mueller, H A.]]
-[[Category: Penalva, M.A.]]
+[[Category: Penalva, M A.]]
-[[Category: Timm, D.E.]]
+[[Category: Timm, D E.]]
-[[Category: Titus, G.P.]]
+[[Category: Titus, G P.]]
 [[Category: beta sandwich]]
 [[Category: jelly roll]]
-''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri Feb 15 15:45:13 2008''
+''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 12:32:49 2008''