1ajj | pdb_00001ajj
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LDL RECEPTOR LIGAND-BINDING MODULE 5, CALCIUM-COORDINATING
Overview
The low-density lipoprotein receptor (LDLR) is responsible for the uptake of cholesterol-containing lipoprotein particles into cells. The amino-terminal region of LDLR, which consists of seven tandemly repeated, approximately 40-amino-acid, cysteine-rich modules (LDL-A modules), mediates binding to lipoproteins. LDL-A modules are biologically ubiquitous domains, found in over 100 proteins in the sequence database. The structure of ligand-binding repeat 5 (LR5) of the LDLR, determined to 1.7 A resolution by X-ray crystallography and presented here, contains a calcium ion coordinated by acidic residues that lie at the carboxy-terminal end of the domain and are conserved among LDL-A modules. Naturally occurring point mutations found in patients with the disease familial hypercholesterolaemia alter residues that directly coordinate Ca2+ or that serve as scaffolding residues of LR5.
Disease
Known disease associated with this structure: Hypercholesterolemia, familial OMIM:[606945]
About this Structure
1AJJ is a Single protein structure of sequence from Homo sapiens with SO4 and CA as ligands. Full crystallographic information is available from OCA.
Reference
Molecular basis of familial hypercholesterolaemia from structure of LDL receptor module., Fass D, Blacklow S, Kim PS, Berger JM, Nature. 1997 Aug 14;388(6643):691-3. PMID:9262405
Page seeded by OCA on Thu Feb 21 11:45:13 2008
