1ajj

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spinqualitylabelsall models 1ajj, resolution 1.7Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image

LDL RECEPTOR LIGAND-BINDING MODULE 5, CALCIUM-COORDINATING

Contents 1 Overview 2 Disease 3 About this Structure 4 Reference

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

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