1op9

From Proteopedia

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Template:STRUCTURE 1op9

Contents 1 Complex of human lysozyme with camelid VHH HL6 antibody fragment 2 Disease 3 Function 4 About this Structure 5 See Also 6 Reference

Complex of human lysozyme with camelid VHH HL6 antibody fragment

Template:ABSTRACT PUBMED 12917687

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

1op9 is a 2 chain structure with sequence from Camelus dromedarius and Homo sapiens. Full crystallographic information is available from OCA.

See Also

• Hen Egg-White (HEW) Lysozyme

Reference

• Dumoulin M, Last AM, Desmyter A, Decanniere K, Canet D, Larsson G, Spencer A, Archer DB, Sasse J, Muyldermans S, Wyns L, Redfield C, Matagne A, Robinson CV, Dobson CM. A camelid antibody fragment inhibits the formation of amyloid fibrils by human lysozyme. Nature. 2003 Aug 14;424(6950):783-8. PMID:12917687 doi:http://dx.doi.org/10.1038/nature01870

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