1hkl

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Template:STRUCTURE 1hkl

Contents 1 FREE AND LIGANDED FORM OF AN ESTEROLYTIC CATALYTIC ANTIBODY 2 Disease 3 About this Structure 4 See Also 5 Reference

FREE AND LIGANDED FORM OF AN ESTEROLYTIC CATALYTIC ANTIBODY

Template:ABSTRACT PUBMED 9159478

Disease

[IGKC_HUMAN] Defects in IGKC are the cause of immunoglobulin kappa light chain deficiency (IGKCD) [MIM:614102]. IGKCD is a disease characterized by the complete absence of immunoglobulin kappa chains.^[1] [IGHG1_HUMAN] Defects in IGHG1 are a cause of multiple myeloma (MM) [MIM:254500]. MM is a malignant tumor of plasma cells usually arising in the bone marrow and characterized by diffuse involvement of the skeletal system, hyperglobulinemia, Bence-Jones proteinuria and anemia. Complications of multiple myeloma are bone pain, hypercalcemia, renal failure and spinal cord compression. The aberrant antibodies that are produced lead to impaired humoral immunity and patients have a high prevalence of infection. Amyloidosis may develop in some patients. Multiple myeloma is part of a spectrum of diseases ranging from monoclonal gammopathy of unknown significance (MGUS) to plasma cell leukemia. Note=A chromosomal aberration involving IGHG1 is found in multiple myeloma. Translocation t(11;14)(q13;q32) with the IgH locus. Translocation t(11;14)(q13;q32) with CCND1; translocation t(4;14)(p16.3;q32.3) with FGFR3; translocation t(6;14)(p25;q32) with IRF4.

About this Structure

1hkl is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

See Also

• Antibody

Reference

• Wedemayer GJ, Wang LH, Patten PA, Schultz PG, Stevens RC. Crystal structures of the free and liganded form of an esterolytic catalytic antibody. J Mol Biol. 1997 May 2;268(2):390-400. PMID:9159478 doi:10.1006/jmbi.1997.0974

1. ↑ Stavnezer-Nordgren J, Kekish O, Zegers BJ. Molecular defects in a human immunoglobulin kappa chain deficiency. Science. 1985 Oct 25;230(4724):458-61. PMID:3931219