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MBL<br />

81051<br />

1 0.35-0.69 Equivocal<br />

2 0.70-3.49 Positive<br />

3 3.50-17.4 Positive<br />

4 17.5-49.9 Strongly positive<br />

5 50.0-99.9 Strongly positive<br />

6 > or =100 Strongly positive Reference values<br />

apply to all ages.<br />

Clinical References: Homburger HA: Allergic diseases. In Clinical Diagnosis and Management by<br />

Laboratory Methods. 21st edition. Edited by McPherson RA, Pincus MR. WB Saunders, Publ, New York,<br />

Chapter 53, Part VI, pp. 961-971, 2007<br />

Mannose-Binding Lectin, Serum<br />

Clinical Information: Mannose-binding lectin (MBL) is a member of the collectin family of proteins<br />

that are characterized structurally by the presence of collagenous regions and lectin domains in the same<br />

subunit. The subunit structure of MBL is comprised of three 32kDa peptide chains organized in a triple<br />

helix with 3 C-terminal lectin domains. Circulating (functional) MBL is comprised of oligomers of<br />

subunits (dimers through tetramers account for approximately 75% of circulating MBL) that are<br />

associated with an MBL-associated serine protease (MASP). There is a single MBL gene (4 exons) on<br />

chromosome 10 with 4 known allelic variants: wild type MBL (A), and 3 mutant forms B, C, and D<br />

caused by point mutations in 3 different codons. The mutant forms of MBL form oligomers poorly, and<br />

have diminished complement activating activity (see below). Multimeric MBL binds to many different<br />

oligosaccharide moieties, including those in the cell walls of many different bacteria, yeasts, and some<br />

viruses, including HIV 1, HIV 2, and influenza A. Binding of MBL results in complement activation by<br />

the classical pathway through activation of complement 4 (C4) by MASP, and surface bound MBL<br />

enhances phagocytosis by interacting with collectin receptors on phagocytic cells. Mutations of MBL<br />

codons (homozygous or heterozygous haplotypes) are associated with diminished opsonophagocytic<br />

activity and diminished serum levels of MBL measured immunochemically (MBL deficiency).<br />

MBL-deficient individuals have increased susceptibility to infection particularly if MBL deficiency<br />

occurs concomittantly with another heritable immune system abnormality, eg, C4 null alleles or<br />

immunoglobulin class or subclass deficiencies.<br />

Useful For: Evaluation of children and adults with a clinical history of recurrent infections Results<br />

may be useful for genetic counseling and support aggressive management of recurrent infections in<br />

patients with reduced levels of mannose-binding lectin<br />

Interpretation: Diminished levels of serum mannose-binding lectin (MBL) are consistent with the<br />

diagnosis of MBL deficiency. Levels or =7.8 ng/mL<br />

This reference range applies only to adults.<br />

See Cautions for further information regarding the reference range and clinical interpretation.<br />

Clinical References: 1. Pettigrew HD, Teuber SS, Gershwin ME: Clinical significance of<br />

complement deficiencies. Ann NY Acad Sci 2009;1173:108-123 2. Botto M, Kirschfind M, Maco P, et al:<br />

Complement in human diseases: lessons from complement deficiencies. Mol Immunol<br />

2009;48:2774-2783 3. Thiel S, Frederiksen PD, Jensenius JC: Clinical manifestations of mannan-binding<br />

lectin deficiency. Mol Immunol 2006;43:86-96<br />

FMPLE<br />

Current 57188 as of January 4, 2013 7:15 pm CST 800-533-1710 or 507-266-5700 or <strong>Mayo</strong><strong>Medical</strong><strong>Laboratories</strong>.com Page 1160

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