1ei9: Difference between revisions

Revision as of 13:28, 21 February 2008

CRYSTAL STRUCTURE OF PALMITOYL PROTEIN THIOESTERASE 1

OverviewOverview

Mutations in palmitoyl-protein thioesterase 1 (PPT1), a lysosomal enzyme that removes fatty acyl groups from cysteine residues in modified proteins, cause the fatal inherited neurodegenerative disorder infantile neuronal ceroid lipofuscinosis. The accumulation of undigested substrates leads to the formation of neuronal storage bodies that are associated with the clinical symptoms. Less severe forms of PPT1 deficiency have been found recently that are caused by a distinct set of PPT1 mutations, some of which retain a small amount of thioesterase activity. We have determined the crystal structure of PPT1 with and without bound palmitate by using multiwavelength anomalous diffraction phasing. The structure reveals an alpha/beta-hydrolase fold with a catalytic triad composed of Ser115-His289-Asp233 and provides insights into the structural basis for the phenotypes associated with PPT1 mutations.

About this StructureAbout this Structure

1EI9 is a Single protein structure of sequence from Bos taurus with and as ligands. Active as Palmitoyl-protein hydrolase, with EC number 3.1.2.22 Full crystallographic information is available from OCA.

ReferenceReference

The crystal structure of palmitoyl protein thioesterase 1 and the molecular basis of infantile neuronal ceroid lipofuscinosis., Bellizzi JJ 3rd, Widom J, Kemp C, Lu JY, Das AK, Hofmann SL, Clardy J, Proc Natl Acad Sci U S A. 2000 Apr 25;97(9):4573-8. PMID:10781062

Page seeded by OCA on Thu Feb 21 12:28:08 2008

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OCA

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-[[Image:1ei9.jpg|left|200px]]<br /><applet load="1ei9" size="450" color="white" frame="true" align="right" spinBox="true"
+[[Image:1ei9.jpg|left|200px]]<br /><applet load="1ei9" size="350" color="white" frame="true" align="right" spinBox="true"
 caption="1ei9, resolution 2.25&Aring;" />
 '''CRYSTAL STRUCTURE OF PALMITOYL PROTEIN THIOESTERASE 1'''<br />
 ==Overview==
-Mutations in palmitoyl-protein thioesterase 1 (PPT1), a lysosomal enzyme, that removes fatty acyl groups from cysteine residues in modified, proteins, cause the fatal inherited neurodegenerative disorder infantile, neuronal ceroid lipofuscinosis. The accumulation of undigested substrates, leads to the formation of neuronal storage bodies that are associated with, the clinical symptoms. Less severe forms of PPT1 deficiency have been, found recently that are caused by a distinct set of PPT1 mutations, some, of which retain a small amount of thioesterase activity. We have, determined the crystal structure of PPT1 with and without bound palmitate, by using multiwavelength anomalous diffraction phasing. The structure, reveals an alpha/beta-hydrolase fold with a catalytic triad composed of, Ser115-His289-Asp233 and provides insights into the structural basis for, the phenotypes associated with PPT1 mutations.
+Mutations in palmitoyl-protein thioesterase 1 (PPT1), a lysosomal enzyme that removes fatty acyl groups from cysteine residues in modified proteins, cause the fatal inherited neurodegenerative disorder infantile neuronal ceroid lipofuscinosis. The accumulation of undigested substrates leads to the formation of neuronal storage bodies that are associated with the clinical symptoms. Less severe forms of PPT1 deficiency have been found recently that are caused by a distinct set of PPT1 mutations, some of which retain a small amount of thioesterase activity. We have determined the crystal structure of PPT1 with and without bound palmitate by using multiwavelength anomalous diffraction phasing. The structure reveals an alpha/beta-hydrolase fold with a catalytic triad composed of Ser115-His289-Asp233 and provides insights into the structural basis for the phenotypes associated with PPT1 mutations.
 ==About this Structure==
-EI9 is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Bos_taurus Bos taurus] with NDG and NAG as [http://en.wikipedia.org/wiki/ligands ligands]. Active as [http://en.wikipedia.org/wiki/Palmitoyl-protein_hydrolase Palmitoyl-protein hydrolase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.1.2.22 3.1.2.22] Full crystallographic information is available from [http://ispc.weizmann.ac.il/oca-bin/ocashort?id=1EI9 OCA].
+EI9 is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Bos_taurus Bos taurus] with <scene name='pdbligand=NDG:'>NDG</scene> and <scene name='pdbligand=NAG:'>NAG</scene> as [http://en.wikipedia.org/wiki/ligands ligands]. Active as [http://en.wikipedia.org/wiki/Palmitoyl-protein_hydrolase Palmitoyl-protein hydrolase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.1.2.22 3.1.2.22] Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1EI9 OCA].
 ==Reference==
@@ Line 15: / Line 15: @@
 [[Category: Single protein]]
 [[Category: Clardy, J.]]
-[[Category: Das, A.K.]]
+[[Category: Das, A K.]]
-[[Category: Hofmann, S.L.]]
+[[Category: Hofmann, S L.]]
-[[Category: III, J.J.Bellizzi.]]
+[[Category: III, J J.Bellizzi.]]
 [[Category: Kemp, C.]]
-[[Category: Lu, J.Y.]]
+[[Category: Lu, J Y.]]
 [[Category: Widom, J.]]
 [[Category: NAG]]
@@ Line 26: / Line 26: @@
 [[Category: glycoprotein]]
-''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Tue Nov 20 14:01:08 2007''
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