1hj7

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[[Image:1hj7.gif|left|200px]]
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[[Image:1hj7.png|left|200px]]
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{{STRUCTURE_1hj7| PDB=1hj7 | SCENE= }}
{{STRUCTURE_1hj7| PDB=1hj7 | SCENE= }}
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'''NMR STUDY OF A PAIR OF LDL RECEPTOR CA2+ BINDING EPIDERMAL GROWTH FACTOR-LIKE DOMAINS, 20 STRUCTURES'''
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===NMR STUDY OF A PAIR OF LDL RECEPTOR CA2+ BINDING EPIDERMAL GROWTH FACTOR-LIKE DOMAINS, 20 STRUCTURES===
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==Overview==
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BACKGROUND: From the observed structure and sequence of a pair of calcium binding (cb) epidermal growth factor-like (EGF) domains from human fibrillin-1, we proposed that many tandem cbEGF domains adopt a conserved relative conformation. The low-density lipoprotein receptor (LDLR), which is functionally unrelated to fibrillin-1, contains a single pair of EGF domains that was chosen for study in the validation of this hypothesis. The LDLR is the protein that is defective in familial hypercholesterolaemia, a common genetic disorder that predisposes individuals to cardiovascular complications and premature death. RESULTS: Here, we present the solution structure of the first two EGF domains from the LDL receptor, determined using conventional NMR restraints and residual dipolar couplings. The cbEGF domains have an elongated, rod-like arrangement, as predicted. The new structure allows a detailed assessment of the consequences of mutations associated with familial hypercholesterolaemia to be made. CONCLUSIONS: The validation of the conserved arrangement of EGF domains in functionally distinct proteins has important implications for structural genomics, since multiple tandem cbEGF pairs have been identified in many essential proteins that are implicated in human disease. Our results provide the means to use homology modeling to probe structure-function relationships in this diverse family of proteins and may hold the potential for the design of novel diagnostics and therapies in the future.
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(as it appears on PubMed at http://www.pubmed.gov), where 11435110 is the PubMed ID number.
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{{ABSTRACT_PUBMED_11435110}}
==Disease==
==Disease==
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==About this Structure==
==About this Structure==
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1HJ7 is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1HJ7 OCA].
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1HJ7 is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1HJ7 OCA].
==Reference==
==Reference==
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[[Category: Module]]
[[Category: Module]]
[[Category: Vldl]]
[[Category: Vldl]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May 2 18:53:52 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 1 08:15:35 2008''

Revision as of 05:15, 1 July 2008

Image:1hj7.png

Template:STRUCTURE 1hj7

Contents

NMR STUDY OF A PAIR OF LDL RECEPTOR CA2+ BINDING EPIDERMAL GROWTH FACTOR-LIKE DOMAINS, 20 STRUCTURES

Template:ABSTRACT PUBMED 11435110

Disease

Known disease associated with this structure: Hypercholesterolemia, familial OMIM:[606945]

About this Structure

1HJ7 is a Single protein structure of sequence from Homo sapiens. Full experimental information is available from OCA.

Reference

Solution structure of the LDL receptor EGF-AB pair: a paradigm for the assembly of tandem calcium binding EGF domains., Saha S, Boyd J, Werner JM, Knott V, Handford PA, Campbell ID, Downing AK, Structure. 2001 Jun;9(6):451-6. PMID:11435110

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