4i0c

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Template:STRUCTURE 4i0c

Contents 1 The structure of the camelid antibody cAbHuL5 in complex with human lysozyme 2 Disease 3 Function 4 About this Structure 5 Reference

The structure of the camelid antibody cAbHuL5 in complex with human lysozyme

Template:ABSTRACT PUBMED 23919586

Disease

[LYSC_HUMAN] Defects in LYZ are a cause of amyloidosis type 8 (AMYL8) [MIM:105200]; also known as systemic non-neuropathic amyloidosis or Ostertag-type amyloidosis. AMYL8 is a hereditary generalized amyloidosis due to deposition of apolipoprotein A1, fibrinogen and lysozyme amyloids. Viscera are particularly affected. There is no involvement of the nervous system. Clinical features include renal amyloidosis resulting in nephrotic syndrome, arterial hypertension, hepatosplenomegaly, cholestasis, petechial skin rash.^[1]

Function

[LYSC_HUMAN] Lysozymes have primarily a bacteriolytic function; those in tissues and body fluids are associated with the monocyte-macrophage system and enhance the activity of immunoagents.

About this Structure

4i0c is a 4 chain structure with sequence from Camelus dromedarius and Homo sapiens. Full crystallographic information is available from OCA.

Reference

• De Genst E, Chan PH, Pardon E, Hsu ST, Kumita JR, Christodoulou J, Menzer L, Chirgadze DY, Robinson CV, Muyldermans S, Matagne A, Wyns L, Dobson CM, Dumoulin M. A Nanobody Binding to Non-Amyloidogenic Regions of the Protein Human Lysozyme Enhances Partial Unfolding but Inhibits Amyloid Fibril Formation. J Phys Chem B. 2013 Sep 24. PMID:23919586 doi:http://dx.doi.org/10.1021/jp403425z

1. ↑ Pepys MB, Hawkins PN, Booth DR, Vigushin DM, Tennent GA, Soutar AK, Totty N, Nguyen O, Blake CC, Terry CJ, et al.. Human lysozyme gene mutations cause hereditary systemic amyloidosis. Nature. 1993 Apr 8;362(6420):553-7. PMID:8464497 doi:http://dx.doi.org/10.1038/362553a0