1jbq

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(New page: 200px<br /> <applet load="1jbq" size="450" color="white" frame="true" align="right" spinBox="true" caption="1jbq, resolution 2.60&Aring;" /> '''STRUCTURE OF HUMAN ...)
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'''STRUCTURE OF HUMAN CYSTATHIONINE BETA-SYNTHASE: A UNIQUE PYRIDOXAL 5'-PHOSPHATE DEPENDENT HEMEPROTEIN'''<br />
'''STRUCTURE OF HUMAN CYSTATHIONINE BETA-SYNTHASE: A UNIQUE PYRIDOXAL 5'-PHOSPHATE DEPENDENT HEMEPROTEIN'''<br />
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==About this Structure==
==About this Structure==
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1JBQ is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with HEM and PLP as [http://en.wikipedia.org/wiki/ligands ligands]. Active as [http://en.wikipedia.org/wiki/Cystathionine_beta-synthase Cystathionine beta-synthase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=4.2.1.22 4.2.1.22] Full crystallographic information is available from [http://ispc.weizmann.ac.il/oca-bin/ocashort?id=1JBQ OCA].
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1JBQ is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with <scene name='pdbligand=HEM:'>HEM</scene> and <scene name='pdbligand=PLP:'>PLP</scene> as [http://en.wikipedia.org/wiki/ligands ligands]. Active as [http://en.wikipedia.org/wiki/Cystathionine_beta-synthase Cystathionine beta-synthase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=4.2.1.22 4.2.1.22] Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1JBQ OCA].
==Reference==
==Reference==
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[[Category: fold type ii of plp enzymes]]
[[Category: fold type ii of plp enzymes]]
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''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Mon Nov 12 17:39:19 2007''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri Feb 15 16:06:39 2008''

Revision as of 14:06, 15 February 2008


1jbq, resolution 2.60Å

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STRUCTURE OF HUMAN CYSTATHIONINE BETA-SYNTHASE: A UNIQUE PYRIDOXAL 5'-PHOSPHATE DEPENDENT HEMEPROTEIN

Contents

Overview

Cystathionine beta-synthase (CBS) is a unique heme- containing enzyme that, catalyzes a pyridoxal 5'-phosphate (PLP)-dependent condensation of serine, and homocysteine to give cystathionine. Deficiency of CBS leads to, homocystinuria, an inherited disease of sulfur metabolism characterized by, increased levels of the toxic metabolite homocysteine. Here we present the, X-ray crystal structure of a truncated form of the enzyme. CBS shares the, same fold with O-acetylserine sulfhydrylase but it contains an additional, N-terminal heme binding site. This heme binding motif together with a, spatially adjacent oxidoreductase active site motif could explain the, regulation of its enzyme activity by redox changes.

Disease

Known diseases associated with this structure: Homocystinuria, B6-responsive and nonresponsive types OMIM:[236200], Thrombosis, hyperhomocysteinemic OMIM:[236200]

About this Structure

1JBQ is a Single protein structure of sequence from Homo sapiens with and as ligands. Active as Cystathionine beta-synthase, with EC number 4.2.1.22 Full crystallographic information is available from OCA.

Reference

Structure of human cystathionine beta-synthase: a unique pyridoxal 5'-phosphate-dependent heme protein., Meier M, Janosik M, Kery V, Kraus JP, Burkhard P, EMBO J. 2001 Aug 1;20(15):3910-6. PMID:11483494

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