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1ei9

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{{STRUCTURE_1ei9| PDB=1ei9 | SCENE= }}
{{STRUCTURE_1ei9| PDB=1ei9 | SCENE= }}
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'''CRYSTAL STRUCTURE OF PALMITOYL PROTEIN THIOESTERASE 1'''
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===CRYSTAL STRUCTURE OF PALMITOYL PROTEIN THIOESTERASE 1===
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==Overview==
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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.
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{{ABSTRACT_PUBMED_10781062}}
==About this Structure==
==About this Structure==
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[[Category: Alpha/beta hydrolase]]
[[Category: Alpha/beta hydrolase]]
[[Category: Glycoprotein]]
[[Category: Glycoprotein]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 1 00:44:30 2008''

Revision as of 21:44, 30 June 2008

Image:1ei9.png

Template:STRUCTURE 1ei9

CRYSTAL STRUCTURE OF PALMITOYL PROTEIN THIOESTERASE 1

Template:ABSTRACT PUBMED 10781062

About this Structure

1EI9 is a Single protein structure of sequence from Bos taurus. Full crystallographic information is available from OCA.

Reference

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

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