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Table 1 Description of inflammation- and fibrosis-associated genes investigated in human (OP and control) lungs and in transgenic and nontransgenic mice

From: Organizing pneumonia in mice and men

Gene name Approved symbol (human/mouse) Approved symbol (mouse) Function
Bone morphogenetic protein 4 BMP4 Bmp4 BMPs are a group of growth factors also known as cytokines and are part of the transforming growth factor beta superfamily. BMPs induce cartilage and bone formation
Bone morphogenetic protein receptor, type 1B BMPR1B Bmpr1b On ligand binding, a receptor complex forms consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators
Chemokine (C-C motif) ligand 2 CCL2 Ccl2 This chemokine is a member of the CC subfamily which is characterized by two adjacent cysteine residues. This cytokine displays chemotactic activity for monocytes and basophils but not for neutrophils or eosinophils. It has been implicated in the pathogenesis of diseases characterized by monocytic infiltrates, like psoriasis, rheumatoid arthritis and atherosclerosis. It binds to chemokine receptors CCR2 and CCR4
Collagen, type III, alpha 1 COL3A1 Col3a1 Collagen type III occurs in most soft connective tissues (skin, lung, uterus, intestine and the vascular system) along with type I collagen. Involved in the regulation of cortical development
Chemokine (C-X-C motif) ligand 12 CXCL12 Cxcl12 Chemoattractant active on T-lymphocytes, monocytes, but not neutrophils. It functions as the ligand for the G protein-coupled receptor, chemokine (C-X-C motif) receptor 4, and plays a role in many diverse cellular functions, including embryogenesis, immune surveillance, inflammation response, tissue homeostasis, and tumor growth and metastasis
Chemokine (C-X-C motif) receptor 4 CXCR4 Cxcr4 Receptor for the C-X-C chemokine CXCL12/SDF-1 that transduces a signal by increasing intracellular calcium ion levels and enhancing MAPK1/MAPK3 activation. Acts as a receptor for extracellular ubiquitin; leading to enhanced intracellular calcium ions and reduced cellular cAMP levels
Interleukin 6 IL6 Il6 Cytokine that functions in inflammation and the maturation of B cells. In addition, it has been shown to be an endogenous pyrogen capable of inducing fever in people with autoimmune diseases or infections. The protein is primarily produced at sites of acute and chronic inflammation, where it is secreted into the serum and induces a transcriptional inflammatory response through interleukin 6 receptor alpha
Matrix metallopeptidase 2 MMP2 Mmp2 Ubiquitinous metalloproteinase that is involved in diverse functions such as remodeling of the vasculature, angiogenesis, tissue repair, tumor invasion, inflammation, and atherosclerotic plaque rupture. As well as degrading extracellular matrix proteins, it can also act on several non-matrix proteins such as big endothelin-1 and beta-type CGRP promoting vasoconstriction
Protein tyrosine kinase 2 PTK2 Ptk2 Non-receptor protein-tyrosine kinase that plays an essential role in regulating cell migration, adhesion, spreading, reorganization of the actin cytoskeleton, formation and disassembly of focal adhesions and cell protrusions, cell cycle progression, cell proliferation and apoptosis
SMAD family member 1 SMAD1 Smad1 This protein mediates the signals of the bone morphogenetic proteins (BMPs), which are involved in a range of biological activities including cell growth, apoptosis, morphogenesis, development and immune responses. In response to BMP ligands, this protein can be phosphorylated and activated by the BMP receptor kinase, type I. The phosphorylated form of this protein forms a complex with SMAD4, which is important for its function in the transcription regulation
SMAD family member 3 SMAD3 Smad3 Receptor-regulated SMAD (R-SMAD) that is an intracellular signal transducer and transcriptional modulator activated by TGF-beta (transforming growth factor) and is thought to play a role in the regulation of carcinogenesis
Transforming growth factor, beta 1 TGFB1 Tgfb1 Multifunctional protein that controls proliferation, differentiation and other functions in many cell types. Many cells synthesize TGFB1 and have specific receptors for it. It positively and negatively regulates many other growth factors. At low concentrations in concert with IL-6 and IL-21, it leads to expression of the IL-17 and IL-23 receptors, favoring differentiation to Th17 cells
Thrombospondin 1 THBS1 Thbs1 This protein is an adhesive glycoprotein that mediates cell-to-cell and cell-to-matrix interactions. It can bind to fibrinogen, fibronectin, laminin, type V collagen and integrins alpha-V/beta-1 and has been shown to play roles in platelet aggregation, angiogenesis, and tumorigenesis
Tissue inhibitor of metalloproteinase 1 TIMP1 Timp1 Metalloproteinase inhibitor that functions by forming one to one complexes with target metalloproteinases, such as collagenases, and irreversibly inactivates them by binding to their catalytic zinc cofactor. Acts on all MMPs, except on MMP14. Also functions as a growth factor that regulates cell differentiation, migration and cell death and activates cellular signaling cascades
Tissue inhibitor of metalloproteinase 2 TIMP2 Timp2 In addition to an inhibitory role against metalloproteinases, this protein has a unique role among TIMP family members in its ability to directly suppress the proliferation of endothelial cells. As a result, the encoded protein may be critical to the maintenance of tissue homeostasis by suppressing the proliferation of quiescent tissues in response to angiogenic factors, and by inhibiting protease activity in tissues undergoing remodeling of the extracellular matrix
  1. Information is taken from HUGO gene Nomenclature Committee, Uniprot and NCBI-Gene