Journal of Clinical and Cellular Immunology
Open Access
ISSN: 2155-9899
ISSN: 2155-9899
Evans J
United Kingdom
Research Article
Deficiency of Mannose Associated Serine Protease2 (MASP2) in Patients with Idiopathic Pulmonary Fibrosis
Author(s): Varney VA, Parnell H, Quirke G, Bansal A, Sumar N, Nicholas A and Evans JVarney VA, Parnell H, Quirke G, Bansal A, Sumar N, Nicholas A and Evans J
Background: Idiopathic pulmonary fibrosis (UIP/IPF) is increasing. The condition occurs both sporadically and in close blood relatives suggesting a genetic predisposition. Histology suggests that the fibrosis is linked to disordered apoptosis, but the causal agent is so far unrecognised. We previously published evidence of mannose binding lectin deficiency (MBL) in early onset disease and in those with a family history of UIP/IPF. MBL is part of the innate immune system with levels genetically determined. MBL activation is impaired if the mannose associated serine protease2 (MASP2) is severely deficient in the serum (levels<100 ng/ml). The MBL/MASP2 complex is involved in phagocytosis, complement activation and clearance of apoptotic cells. Levels of MASP2 are genetically determined and not influenced by inflammation. Aims.. View More»
DOI:
10.4172/2155-9899.1000331