NOVEL THERAPEUTIC DRUGS & TARGETS FOR ASTHMA

The mainstay of current drug therapy for asthma is the combination of inhaled corticosteroids with long-acting2-agonists (LABAs).. Several improved inhaled corticosteroids, longer-acting LABAs and LAMAs (and combinations) are now in clinical development.  The first biologic agent to be licensed for the treatment of severe allergic asthma is a monoclonal antibody directed against IgE (anti-IgE), while a variety of approaches involving allergen immunotherapy, T cells and pattern recognition receptors are being developed. Specifi c biological antagonists of individual cytokines (IL-5, IL-4, IL-13, IL-9, TNF) have generally been disappointing in asthma, although anti-IL-5 has recently been found to be effective in various conditions with high levels of eosinophils and for severe eosinophilic asthma. Oral CCR3 antagonists have been studied in mild eosinophilic asthma, while in more severe neutrophilic asthma and COPD, therapies such as CXCR2 antagonists, p38 mitogen-activated protein kinase, nuclear factor-B and phosphoinositide 3 kinase inhibitors are being developed. Cilomilast (Arifl o, GlaxoSmithKline) and roflumilast (Daxas, Nycomed/ Merck) are oral inhibitors of the enzyme phosphodiesterase type 4 and have been assessed in large phase III studies, and roflumilast is under regulatory review by the European Medicines Agency.  Amongst the new generation of nucleic acid-directed therapies, inhaled anti-sense and small-interfering RNA approaches have recently been assessed in asthma. Hence, as a result of the considerable ongoing efforts by clinicians, scientists and the pharmaceutical industry to develop new therapies for asthma andCOPD, progress is gradually being made.

 BRONCHODILATORS


1.INDACETROL: A NEW ONCE DAILY LONG ACTING β2ADRENOCEPTOR AGONIST (LABA)
Indacaterol is a novel once-daily long-acting -adrenoceptor agonist in development for the treatment of obstructive airway disease, namely chronic obstructive pulmonary disease (COPD) and (in combination withan inhaled corticosteroid) asthma. Clinical studies suggest that indacaterol produces rapid and sustained bronchodilation in patients with COPD or asthma in a range of different severities. Until now, clinical studies (PHASE 2) with durations of up to 28 days have been published, and these studies confirmed suitability of indacaterol for once-daily dosing, along with a favorable overall safety and tolerability profile.

2
.ACLIDINIUM BROMIDE: A NOVEL LONG-ACTING MUSCARINIC ANTAGONIST (LAMA)
Aclidinium bromide (Almirall/Forest, previously LAS-34273), a novel, long-acting muscarinic antagonist, administered via a new multidose dry powder inhaler, is currently (March 2008) in phase III development for the maintenance treatment of patients with chronic obstructive pulmonary disease (COPD) and asthma. Preclinical studies have demonstrated that aclidinium is a potent, competitive muscarinic receptor antagonist with kinetic selectivity for M3over M2receptors. Aclidinium has a long residency at M3receptors and results of preclinical and early clinical studies in healthy subjects indicate that aclidinium provides long-lasting protection against bronchoconstriction. This has been confirmed in a phase IIa study of patients with moderate to severe COPD where aclidinium produced sustained bronchodilation over 24 hours, demonstrating suitability for once-daily dosing. In human plasma, aclidinium is rapidly hydrolyzed to two major inactive metabolites. The very low and transient systemic exposure to aclidinium is consistent with an excellent tolerability profile, as demonstrated thus far in the clinical trial program.
3.
DUAL-PHARMACOLOGY BRONCHODILATORS
        Inhaled bronchodilators such β2-adrenoceptor agonists (BAs) and muscarinic receptor antagonists (MAs) are commonly used in the treatment of chronic obstructive pulmonary disease (COPD). Combinations comprised of one agent from each group are recommended for aggressive treatment of more severe disease.  Dual pharmacology, where a single small molecule is able to act via two pharmacological mechanisms, is a novel approach to bronchodilation that could provide equivalent or possibly superior treatment for patients with COPD. Compounds with both MA and BA activity (MABAs) offer a single pharmacokinetic profile for both pharmacological activities, potential for maximizing the synergy between the two mechanisms, and a simpler technical and clinical development pathway compared to co-formulation of two compounds. In addition, the MABA approach may offer a unique opportunity for combination with an antiinflammatory drug (such as an inhaled corticosteroid) to provide “triple therapy” in a single inhalation device. Several prototypic MABA molecules, including GSK-961081 (TD-5959), are in preclinical or clinical phases of development.

IgE- AND  EPITHELIAL-DIRECTED APPROACHES

4. IgE-directed approaches: A general strategy for the treatment of atopic diseases
Immunoglobulin E (IgE) is now generally recognized as a key mediator in the pathogenesis of atopic diseases such as allergic asthma and rhinitis. Therapeutic strategies targeting IgE resulted in the development of humanized neutralizing monoclonal anti-IgE antibodies. They attach to an epitope which overlaps with a region within the IgE molecule that also interacts with its high- and low-affinity receptors expressed on mast cells, basophils and dendritic cells. Thus, these anti-IgE antibodies have the capacity to bind to and neutralize free serum IgE and membrane-bound IgE on B cells but do not recognize receptor-bound IgE.  Omalizumab (Xolair®) is the most advanced therapeutic anti-IgE antibody. By removing free IgE from the circulation it also markedly downregulates the density of high-affinity IgE receptors on the surface of effector cells. After having been studied in more than 20 phase II and phase III clinical trials for various atopic indications, omalizumab has been approved to treat patients with moderate to severe asthma.
5.Therapy directed against thymic stromal lymphopoietin (TSLP)
Thymic stromal lymphopoietin (TSLP) is an interleukin (IL)-7-like cytokine and has been shown to be one of the factors released by epithelial cells following allergen contact with an important role instructing dendritic cells (DCs) to induce a T-helper type 2 (Th2) response. These TSLP-DC stimulate CD4+T cells to induce a proallergic cytokine profile, suggesting TSLP plays a crucial role in the initiation of the allergic cascade. Recently, evidence has also accumulated that TSLP could play a role in enhancement of the effector stages of the allergic response, with TSLP in synergy with IL-1 and tumor necrosis factor (TNF)- shown to amplify cytokine secretion from mast cells. Also, the clinical relevance of TSLP has been demonstrated by both high levels of TSLP in skin biopsies from lesional atopic dermatitis patients and also increased expression of TSLP in asthmatic airway epithelial cells which correlated with reduced lung function. These studies suggest a critical role for TSLP as a driving factor in the emerging concept of tissue-specific control of immunity with TSLP secretion at the epithelial–DC interface acting as an initial factor in the proallergic cascade.

T-cell co-stimulator blockade

6.ICOS and B7RP-1: Novel targets for monoclonal antibodies
The inducible co-stimulatory molecule (ICOS) receptor is one of several co-stimulators expressed on T cells. ICOS interacts with a distinct cell surface ligand, B7 related protein-1 (B7RP-1 or ICOSL), to regulate Th1- and Th2-dependent immune responses, as well as T-dependent B-cell activation. ICOS expression is increased on T cells from patients with a variety of autoimmune diseases and is linked to increased levels of proinflammatory cytokines. Thus, blocking ICOS/ICOSL signaling is a promising new approach for therapeutic intervention in a number of diseases, including asthma.

PATTERN RECOGNITION RECEPTORS

7.Toll-like receptor 2 as therapeutic target in lung disease
Toll-like receptor 2 (TLR2) is activated by pathogens and nonpathogen insults leading to innate immune responses and inflammation. TLR2 is a promiscuous receptor being activated by a variety of pathogen- associated molecular patterns (PAMPs), but especially by those expressed by Gram-positive bacteria. TLR2 functions in heterodimers with either TLR1 or TLR6, and there is some evidence for tissue-specific preferences for TLR1 versus TLR6 in the activation of TLR2-mediated responses. Activation of TLR2 has now been implicated in a number of respiratory-based conditions including asthma and chronic obstructive pulmonary disease.
8. Toll-like receptor 3
Toll-like receptors (TLRs) form a key part of innate immunity as they detect conserved microbial pathogen-associated molecular patterns and trigger theproduction of proinflammatory cytokines and interferons leading to pathogen elimination. TLR3 is an important member of this family as it detects double-stranded RNA of viral origin and therefore TLR3 function is important in the outcome to viral infection.  Intriguingly, in some viral infections, TLR3 contributes to the pathogenesis of infection, either by using TLR3 for cell entry or by the deleterious effects of excessive proinflammatory cytokines. However, in other viral infections, TLR3 mediates an important host antiviral response. Given their central role in mediating inflammation, TLRs have emerged as exciting therapeutic targets in a number of disease states such as cancer, sterile inflammation and autoimmunity, in addition to the use of TLR agonists as adjuvants. The  therapeutic opportunities in both cancer and in particular respiratory viral infection where TLR3 may be manipulated to provide a clinical benefit.
9.Toll-like receptor 4
The biology of Toll-like receptor 4 (TLR4) is extremely complex. Signaling via endotoxins or endogenous molecules revealed by tissue damage can drive inflammatory responses and have profound effects on the establishment and nature of adaptive immunity. Depending on the context and desired outcome, agonists and antagonists of TLR4 are likely to serve important roles, and safe therapeutics achieving both of these pharmacological aims have been developed. TLR4 agonists are now incorporated as adjuvants in licensed therapeutics; the final place of TLR4 antagonists has yet to be determined.

10.Toll-like receptor 7 and 8 agonists
The Toll-like receptors (TLR) 7 and 8 are mediators of the innate immune response to viral infections. They recognize single-stranded RNAs, but also a variety of small-molecular-weight compounds, such as the imidazoquinoline compounds imiquimod and resiquimod, as well as guanosine analogues and adenine derivatives. While TLR7 is predominantly expressed in plasmacytoid dendritic cells (pDC) and B lymphocytes, TLR8 is mainly expressed by myeloid dendritic cells and monocytes. Activation of TLR7 by agonists induces production of interferon-(IFN-) by pDCs, but also inflammatory cytokines, such as interleukin-12 (IL-12),tumor necrosis factor-              (TNF-), IFN-, and also IL-10. TLR8 activation results mainly in the induction of inflammatory cytokines such as IL-12, TNF-and IFN-. Moreover, agonists can induce dendritic cell maturation and B-cell proliferation. Allergic asthma is characterized by a biased Th2-type immune response, and thus activation of TLR7/8 could be effective in treatment of atopic disease by modulation towards a Th0/Th1-type immune response. Indeed, in several preclinical animal models it was demonstrated that TLR7/8 activation by an agonist can inhibit development of experimental asthma, and cause a shift from a Th2-type towards a Th0/Th1-type immune activation.
11.TOLL-LIKE RECEPTOR 9 ACTIVATION WITH CpG OLIGO DEOXY NUCLEOTIDES FOR ASTHMA THERAPY

Prokaryotic DNA has long been recognized as immunostimulatory. In the last decade the role played by CpG motifs (nucleotide sequence motifs centered on a cytosine-guanine dinucleotide) in bacterial and viral DNA has been elucidated. CpG motifs are detected by the innate immune pattern recognition receptor Toll-like receptor (TLR) 9, the ligation of which activates multiple signal cascades in responding cells. A restricted pattern of TLR9 expression to certain dendritic cells and B cells appears to provide relative specificity in responses, especially in comparison to other TLR ligands. TLR9 activation induces aTh1-like pattern of cytokine release which led to interest in the use of synthetic CpG oligodeoxynucleotides (CpG ODN) for the prevention and treatment of Th2-associated atopic disorders such as asthma. Interestingly, Th1 cytokines do not appear to be necessary for  a therapeutic response in preclinical models of atopic asthma. Additional potential mechanisms of action include induction of regulatory-type responses (involving interleukin-10 release), and expression of indoleamine 2,3-dioxygenase. CpG ODN have been shown to prevent and reverse antigen-induced eosinophilic airway inflammation in animal models; human trials are ongoing with encouraging early results when used as a ragweed vaccine adjuvant in allergic upper airway disease.

Cytokines

12.IL-5-directed approaches in the treatment of eosinophil-driven disease
Eosinophils are involved in the initiation and propagation of diverse inflammatory responses that may result in tissue damage and dysfunction. A number of diseases such as eosinophilic bronchitis, hypereosinophilic syndrome, eosinophilic esophagitis and eosinophilic gastroenteritis are currently considered to be predominantly eosinophil-driven. In atopic disease, their role has been less clear. Eosinophils are critically dependent on the cytokine interleukin-5 (IL-5), for their maturation in bone marrow, and also influence eosinophil migration and survival. Recent clinical data with monoclonal antibodies (mAbs) directed against IL-5 support this assumption. Mepolizumab (SB-240563, GlaxoSmithKline) is a humanized mouse anti-human IL-5 mAb and has been shown to be safe and effective in the treatment of patients with hypereosinophilic syndrome. Most recently, clinical trials in severe asthma and nasal polyposis have also reported positive data increasing our understanding of the role eosinophils play in these disorders.
13.Monoclonal antibody therapy directed against interleukin-5 receptor a: MEDI-563
 MEDI-563, which has also been known as BIW-8405, is a humanized anti-human IL-5 receptor a (IL-5Ra) antibody that not only inhibits the interaction of IL-5 with its high-affinity receptor, but also depletes IL-5Ra-expressing cells through enhanced antibody-dependent cell-mediated cytotoxicity.  Therefore, quantitative depletion of eosinophils and basophils in bone marrow, blood and lung tissue by MEDI-563 might result in enhanced clinical efficacy in diseases characterized by eosinophilic and/or basophilic inflammation.
14.IL-4- and IL-13-directed approaches
Interleukin 4 (IL-4) and IL-13 mediate important proinflammatory functions in asthma including Th2 lymphocyte differentiation, induction of IgE production, upregulation of IgE receptors, expression of vascular cell adhesion molecule-1, promotion of eosinophil transmigration into the lungs, inhibition of T-lymphocyte apoptosis and mucus hypersecretion. The role of IL-4 and IL-13 in the pathogenesis of asthma is further supported by identification of polymorphisms linked to asthma in the IL-4 and IL-13 promoters, the genes themselves and proteins involved in signaling for the two cytokines. Several approaches to IL-4 and IL-3  antagonism are or have been in clinical development.
15.Aerovant (recombinant human interleukin-4 variant)
Aerovant is a recombinant interleukin (IL)-4 variant that is in clinical development for the treatment of asthma and other allergic disease based on its ability to inhibit both IL-4 and IL-13. Phase II studies of Aerovant demonstrate inhibition of late-phase response to allergen challenge in subjects with asthma. The clinical evidence is strong supportive evidence for a continued interest in the contribution of Th2 immunity to the pathogenesis of allergic asthma.
16.An anti-interleukin-13 receptor α-1 antibody for the treatment of asthma
A key role for the interleukin (IL)-13 pathway in asthma and other allergic diseases has been supported by multiple animal models and by consistent genetic associations of IL-13 and its receptor to atopy and asthma in several patient populations. These findings strongly suggest that blocking the IL-13 pathway could be used as a therapeutic approach for the treatment of asthma. Targeting the individual cytokine or components of its receptor complex are currently being explored by a number of companies in order to block this pathway.
17.Interleukin-7-directed approaches
Interleukin-7 (IL-7) is a cytokine involved in T-cell development in the thymus and in peripheral T-cell homeostasis. IL-7 mediates its function through a membrane-bound receptor composed of the common J chain (CD132) and the IL-7-specific D chain (IL-7RD, CD127). The common J chain (CD132) is shared by a number of other cytokine receptors, and the IL-7RD                chain is also used by the receptor for thymic stromal-derived lymphopoietin (TSLP). The TSLP cytokine is involved in the thymic differentiation of regulatory T cells and peripheral modulation of the Th1/Th2 balance. Others and we have identified five polymorphisms in the gene encoding the IL-7RD chain. Four of these single nucleotide polymorphisms (SNPs)—rs1494558 (exon 2), rs1494555 (exon 4), rs6897932 (exon 6) and rs3194051 (exon 8)—give rise to amino acid substitutions, and we have shown significant association with two SNP alleles, rs1494555 and rs6897932, in inhalation allergic patients. The same risk alleles have been associated with survival after allogeneic stem cell transplantation and multiple sclerosis, respectively. The functional impact of IL-7RD polymorphisms is considered an important step towards the development of new treatments based on the modulation of IL-7 and TSLP activities.
18.Monoclonal antibody therapy directed against interleukin-9: MEDI-528
With the identification of T helper 2 (2)-derived cytokines, including interleukin (IL)-9, as orchestrators of allergic asthma, the profound impact of IL-9 on both immune cells and structural cells of the lung has received great attention. Both IL-9 and IL-9 receptor expression are upregulated in human asthmatics, and genetic linkage studies have associated IL-9 with the susceptibility to develop airway hyperresponsiveness. Moreover, preclinical studies highlighting the mast cell-enhancing activity of IL-9, which is unique among the 2 cytokines, indicate that IL-9 blockade could provide a novel approach to the treatment of both noneosinophilic asthma, which is resistant to inhaled corticosteroid therapy, as well as of eosinophilic asthma. MEDI-528 is a humanized immunoglobulin G1 antibody that inhibits the activity of IL-9 and is currently in clinical development, undergoing evaluation in phase IIa studies in mild to moderate asthmatics. MEDI-528 is currently the only program in clinical development directly targeting the IL-9-dependent pathway and may have important therapeutic possibilities in allergic and nonallergic inflammatory diseases where mast cell contributions are prominent.
19.Targeting interleukin-17 in the lungs
A growing body of experimental evidence suggests that interleukin (IL)-17 (also termed IL-17A) is aproinflammatory cytokine uniquely positioned at the interface of innate and adaptive immunity in mammals. Currently believed to be predominantly produced by a unique T-helper (h) subset, the Th17 cells, IL-17 exerts its effects on components of the innate immune system by contributing in direct as well as indirect (i.e.,through inducing the release of other, neutrophil-mobilizing cytokines) ways to the accumulation of inflammatory cells. Thus, IL-17 has been recognized as an important component of the pulmonary host defense in mice and it has been implicated in lung disorders such as bronchial asthma, lung allograft rejection and cystic fibrosis in humans. In addition, recent experimental studies in mouse models of autoimmune diseases have forwarded evidence for an important role of IL-17 in the pathogenesis of these disorders. Unfortunately, there are currently no published clinical trials of the concept of targeting IL-17 in lung disorders. In contrast, there are published clinical trials of blocking IL-23, an upstream regulator of IL-17, in patients with inflammatory bowel disease and psoriasis and these have shown promising results in terms of potentially antiinflammatory effects. Therefore, it now seems motivated to target IL-17 in human lung disorders such as severe asthma, lung allograft rejection and, possibly, even in cystic fibrosis, when conducting new clinical trials.

CHEMOKINES

20.Chemokine CCR3 antagonists
CCR3 expression was first thought to be limited to eosinophils, in vitro studies have now demonstrated that CCR3 is more widely expressed on cells involved in inflammation, such as basophils, mast cells, airway epithelium, smooth muscle cells and T-helper type 2 (Th2) T lymphocytes. Preclinical studies have established that CCR3 antagonism can be used to control allergic inflammation.  Patents and literature indicate that major pharma ceutical companies are still active preclinically in this area and the discovery of second-generation compounds with which to test robustly the therapeutic potential of CCR3 antagonists is eagerly anticipated.
21.Therapeutic potential of CCR4 antagonists
CCR4 is a CC-chemokine receptor which is expressed on a number of T-cell subsets. Inhibition of this receptor is of potential interest as a target in allergic diseases due to its selective expression on Th2 but not Th1 cells and hence the potential to selectively inhibit the recruitment of Th2 cells to sites of allergic inflammation while leaving other immune responses unaffected. CCR4 is also expressed on regulatory T cells and this has led to the hypothesis that CCR4 blockade or a CCR4-directed cytotoxic antibody may be effective in treating certain .

ADHESION MOLECULES

22.Very late activation antigen-4 (VLA-4) antagonists
The integrin VLA-4 (very late activation antigen-4) is a cell surface receptor that mediates cellular adhesion events crucial to leukocyte trafficking and activation.  Although results from human clinical trials using inhaled formulations of small-molecule VLA-4 antagonists have been disappointing to date, a recent trial using an orally available antagonist has proved promising. Natalizumab (Tysabri®), a humanized monoclonal antibody directed against the D4integrin subunit of VLA-4, was successful in clinical trials of multiple sclerosis (MS) and has been approved for this indication. Although natalizumab has provided crucial validation of VLA-4 as a therapeutic target in humans, it has yet to be evaluated in any respiratory indication. Clinical trials with natalizumab also unveiled a potential serious side effect of VLA-4 blockade as three patients were diagnosed with progressive multifocal leukoencephalopathy following treatment. The effectiveness of natalizumab in clinical trials has led many in the pharmaceutical industry to target MS rather than asthma as the primary indication for small-molecule antagonists. As more drugs targeting VLA-4 reach the market, asthma will likely be reconsidered as a potential alternative indication.


23.Selectin antagonists and their potential impact for the treatment of inflammatory lung diseases
Selectin antagonists comprise a heterogeneous group of synthetic molecules, natural product compounds and biologics. They are targeted against the selectin family of cell adhesion molecules (selectins), which are located on the surface of endothelial cells (E- and P-selectin), platelets (P-selectin) and of leukocytes (L-selectin) Selectins play a crucial role in the adhesion and activation of leukocytes and platelets in inflammation. They are supposed to be involved in the pathogenesis of several respiratory diseases such as asthma and chronic obstructive pulmonary disease (COPD). Selectin antagonists targeting one, two or all three selectins (pan- selectin antagonists) were developed. Depending on the target binding site, sialyl Lewis X (sLeX)-mimicking antagonists can be distinguished from antagonists mimicking more complex natural ligand structures like, for example, P-selectin glycoprotein ligand-1 (PSGL-1). To date, the most advanced pan-selectin antagonist in development is Bimosiamose mimicking both sLeX and PSGL-1. It has been demonstrated to be effective in animal models of inflammatory lung diseases and human clinical trials for asthma and COPD.

MEDIATOR RECEPTOR ANTAGONISTS

24.Histamine H4receptor antagonistsate
The histamine H4receptor (H4R) is expressed on multiple cell types involved in the pathology of asthma. Emerging preclinical data in animal models of allergic airway inflammation suggest that H4R antagonists may have a therapeutic effect against inflammation, and against pathological remodeling and changes in airway function. Novel aspects of H4R biology suggest it may also contribute to the etiology of other airway disorders such as chronic obstructive pulmonary disease and cough. Consequently, H4R antagonists are being actively pursued by the pharmaceutical and biotech industry as novel therapies for airway diseases.
25.Antagonists of the prostaglandin D2receptor CRTH2
Prostaglandin D2(PGD2) is produced by mast cells, Th2 lymphocytes and dendritic cells and causes activationOx of Th2 lymphocytes, eosinophils and basophils through a high-affinity interaction with the G protein-coupled receptor chemo attractant homologous receptor expressed on Th2 cells (CRTH2, also known as DP2). Activation of CRTH2 induces chemotaxis of Th2 lymphocytes and eosinophils and has the unusual property of promotingTre cytokine production by Th2 lymphocytes in the absence of allergen or co-stimulation. The ability of supernatants from immunologically activated mast cells to activate Th2 cells and eosinophils is mediated by CRTH2. This receptor also plays an important role in amplifying allergic responses through paracrine activation -80of Th2 cells. Pharmacological blockade or genetic ablation of CRTH2 is associated with a reduction in airways inflammation and reduced levels of mucus, Th2 cytokines and immunoglobulin E. The central role played by CRTH2 in mediating these effects suggests that antagonism of this receptor is an attractive approach to the treatment of chronic allergic disease.

ENZYME INHIBITORS

26.5-Lipoxygenase inhibition
The 5-lipoxygenase (5-LO) is a key arachidonic acid metabolizing enzyme that generates leukotrienes and other eicosanoids capable of producing a variety of potent biological functions observed in asthma and other inflammatory diseases. In spite of the high level of scientific information indicating a prominent role for the 5-LO pathway in chronic inflammatory diseases, there is no inhibitor available to effectively test the hypotheses in the clinic. Novel, nonredox, or iron-ligand chelators and rather competitive inhibitors of 5-LO are needed. These agents should provide good efficacy and safety in asthmatics and they may have a great potential when combined with steroids. Also, new 5-LO inhibitors may help alleviate patients with a variety of inflammatory diseases.
27.Utility of cytosolic phospholipase A2D (cPLA2D) inhibitors in the treatment of asthma
Cytosolic phospholipase A2α (cPLA2α, PLA type IVA) hydrolyzes arachidonyl phospholipids to initiate the production of prostaglandins and leukotrienes and generate the lysophospholipid precursor to platelet activating factor (PAF). Leukotriene antagonists are approved for the treatment of asthma, and both CRTH2, the receptor for prostaglandin D2(PGD2), and PAF receptor have been implicated in asthma. Thus, the concurrent blockade of prostaglandins, leukotrienes and PAF signaling due to a cPLA2α antagonist could be beneficial. However, PGE2, another downstream product of cPLA2α               may be beneficial in asthma. Wyeth has advanced compounds to phase II clinical trials to evaluate safety and efficacy of cPLA2αinhibitors in the treatment of osteoarthritis. These inhibitors are also active in preclinical models of asthma.

ANTIOXIDANTS

28.Antioxidant therapeutic strategies
In view of the evidence implicating oxidative stress in the pathogenesis of several respiratory diseases, one rational approach would be to consider antioxidant intervention in order to neutralize the increased oxidative stress, and the subsequent pathology. Several small-molecular-weight compounds that target oxidant signaling, quench oxidants and the subsequent reactive aldehydes/carbonyls or boost antioxidant potential in the lung are currently being tested clinically. Antioxidant agents such as thiol molecules (glutathione/cysteine and mucolytic drugs, such as N-acetyl-L-cysteine and nacystelyn), dietary polyphenols (curcumin, resveratrol, greentea, quercetin), erdosteine, fudosteine and carbocysteine lysine salt, have all been reported to increase intracellular thiol antioxidant levels and induction of glutathione biosynthesis genes.

PROTEASE INHIBITORS

29.Small-molecule neutrophil elastase inhibitors as therapies for respiratory disease
There is significant evidence in the literature to support the concept that inhibition of neutrophil elastase (NE) will provide antiinflammatory activity, effects on lung remodeling and mucus hypersecretion, and in addition have a positive impact on host defense. Therefore, the potential utility of NE inhibitors in respiratory disease is broad and includes chronic obstructive pulmonary disease, cystic fibrosis and severe asthma. In addition, NE inhibitors could provide a replacement therapy for D1-antitrypsin deficiency. Inhibition of NE should achieve an impact on these diseases through its multifaceted profile which enhances its probability of success in the clinic. The potential of oral and inhaled NE inhibitors will be elucidated in the next decade.

30.The role of MMP-9 and MMP-12 inhibitors in inflammation
Since the discovery of the first matrix metalloproteinase (MMP), this ever growing family of proteinases has been the subject of intense research. Although it was initially believed that MMPs were solely involved in the turnover and degradation of the extracellular matrix, there is now data suggesting MMPs act on cytokines, chemokines and protein mediators to regulate various aspects of inflammation and immunity. For these reasons, there has been great interest in the development of MMP inhibitors as novel therapeutics for the treatment of inflammatory diseases such as asthma and chronic obstructive pulmonary disease.

31. α1-Antitrypsin therapyt
Currently, there are a number of approved therapies for the treatment of panacinar emphysema, a disease that results from severe deficiency of circulating D1-antitrypsin (AAT). The adoption of AAT therapy in the treatment of hereditary emphysema and the well-known activity of AAT as an inhibitor of human neutrophil elastase (HNE) has suggested its use in other indications where -133unregulated HNE may be a causative factor. These include cystic fibrosis, chronic lung disease of prematurity and other, more common forms of chronic obstructive pulmonary disease, such as smoking-related . Other inhibitory pathways that are known for AAT have suggested its further potential in asthma therapy. A growing understanding of global mechanisms involved in respiratory disease progression, together with advances in the inhalation delivery of AAT, suggest that AAT therapy may expand beyond its current use as an infusion therapy for AAT deficiency.

INHIBITORS OF FIBROSIS AND VESSELS

32.Proteinase-activated receptor-1 inhibitors
Proteinase-activated receptor-1 (PAR1) is the major high-affinity thrombin receptor and belongs to a unique family of seven transmembrane domain G-coupled receptors. The discovery of this family of receptors heralded the establishment of a new paradigm for ligand-receptor interaction mechanisms leading to cellular signaling in that these receptors are activated by proteolysis rather than classical ligand binding. PAR1is widely distributed  in the human lung, and recent evidence indicates a role for this receptor in the pathophysiology of lung inflammatory and fibrotic responses. The recent development of potent orally available PAR1antagonists may provide novel opportunities for therapeutic intervention in conditions, such as acute lung injury/acute respiratory distress syndrome and pulmonary fibrosis.
33.Small-molecule inhibitors of transforming growth factor-E type I receptor kinase (ALK5)
Transforming growth factor Es (GF-E1, TGF-E2 and TGF-E3) are members of a large superfamily of growth factors required for normal biological functions. As a consequence of this pleitrophy, dysregulation of this inhibiting TGF-E signaling has become an attractive strategy for drug discovery. There are several modes of inhibiting TGF-E signaling: reducing the production of TGF-E using antisense technology (1), inhibiting the extracellular activation of latent TGF-E by blocking integrin-mediated activation , and sequestering circulating TGF-E using soluble TGF-E receptor or monoclonal antibodies. In addition, a major effort has been invested in inhibiting receptor-mediated signaling using small-molecule inhibitors of the TGF- E type I receptor (TGF-ERI) kinase.
34. Serum amyloid P: A novel antifibrotic agent with therapeutic potential
Serum amyloid P (SAP) is a highly conserved, naturally circulating serum protein and a soluble pattern recognition receptor of the innate immune system. The unique binding activities of SAP suggest that it may localize specifically to sites of injury and function to aid in the removal of damaged tissue. Recent discovery of its ability to regulate certain monocyte differentiation states has identified SAP as a novel and potentially powerful antifibrotic agent, which warrants testing in human clinical trials.

INHIBITORS OF CELL SIGNALING AND TRANSCRIPTION

35.Phosphodiesterase type 4 (PDE4) inhibition:
Phosphodiesterase type 4 (PDE4) inhibition remains a seductive target for new drugs for treatment of a variety of diseases. In many of the cells thought to play a central role in the pathology and symptomatology of asthma, chronic obstructive pulmonary disease and allergic rhinitis, increases in the level of intracellular cAMP leads to suppression of a range of unwanted activities. Since hydrolysis of this key intracellular mediator is controlled primarily by the action of a family of PDE4 isoenzymes, suitable inhibitors should deliver the sought-after efficacy. However, oral agents, including those selective for one isoform or another, have been hampered by mechanism-based adverse effects. Furthermore, inhaled agents though well-tolerated have not delivered the promised benefits. We consider what has been learned from decades of research on PDE4, and consider whether there is promise for new PDE4 inhibitors.

36. Therapy directed against c-kit (CD117) and PDGF transmembrane receptor tyrosine kinases
Imatinib, an oral agent licensed for use in Philadelphia chromosome-positive chronic myeloid leukemia and a variety of other rare conditions, has activity against a range of receptor and nonreceptor tyrosine kinases. C-kit (CD117) stem cell factor receptor and platelet-derived growth factor receptor are transmembrane receptor tyrosine kinases (TMTKs) that are inhibited by imatinib; inhibition of these TMTKs is expected to modulate mast cells, eosinophils and airway remodeling. There is a rationale for the use of imatinib in the treatment of severe allergic and asthmatic disease, and "proof of concept" and therapeutic clinical trials in humans are required. In addition, a variety of TMTK inhibitors are in clinical development, and nisotinib and dasatinib represent compounds of interest.

37.Syk kinase inhibitors
 One of the most important kinases in the IgE signaling pathway is spleen tyrosine kinase (Syk) which has a critical function early in the signaling cascade following binding of allergen to receptor bound IgE on the mast cell. A number of Syk inhibitors have now been developed which have been shown to effectively inhibit IgE-driven mast cell degranulation and release of inflammatory cytokines in vitro and inhibit allergic responses in a variety of in vivo models. In humans, allergen-driven symptoms were reduced in allergic rhinitic patients exposed to tree pollen in an outdoor environment following intranasal dosing of the Syk inhibitor R-112. Syk therefore represents a promising target for therapeutic intervention in allergic diseases.

38.Targeting protein kinase C-T for asthma
Protein kinase C-T (PKC-T)  regulates T-cell receptor (TCR) signaling to interleukin-2 production, with effects on T-cell effector function but not T-cell development. Using mice deficient in PKC-T, a critical role has been defined in Th2-mediated pulmonary inflammation and airway hyperresponsiveness. PKC-T also regulates Th1 cells in experimental autoimmune encephalomyelitis and other models of T-cell-mediated autoimmune diseases. Its selective role in T-cell effector function and the TCR signalosome makes PKC-α an attractive therapeutic target in T-cell-mediated disease processes. PKC-T is also expressed in mast cells and may play a role in IgE-mediated signaling.

NUCLEIC ACID THERAPY

39.A multitargeted antisense therapy directed at CCR3 and the common E-chain of IL-3/IL-5/GM-CSF
TPI ASM8 is an inhaled drug designed to reduce the recruitment and persistence of chronic inflammatory cells in allergic asthma. TPI ASM8 consists of two RNA-targeted, modified antisense oligonucleotides directed at the CCR3 chemokine receptor and the common E-subunit of the interleukin (IL)-3, IL-5 and granulocyte macrophage-colony stimulating factor (GM-CSF) receptors. TPI ASM8 has a unique mechanism of action and represents a novel, highly effective approach for respiratory diseases.

40.Antifungal therapy

With  up  to  60%  of  patients  with  asthma  being  atopic  to common  aeroallergens,  sensitization  to  fungi  appears  to be  an emerging phenotype conferring an increased risk of hospital. Several trials in patients with allergic bronchopulmonary aspergillosis have established the role of antifungal therapy, but only recently have trials in asthma been undertaken. The Fungal Asthma Sensitization Trial (FAST)95 studied patients with severe asthma who were sensitized by a skin prick or radio allergosorbent testing to one or more fungal allergens and did not fulfill the criteria for allergic bronchopulmonary mycosis. Treatment  with  oral  itraconazole  200  mg  twice daily/placebo for 32 weeks resulted in clinically significant improvements  of  asthma  quality  of  life  scores  as  well  as rhinitis and morning peak flows.
              Interestingly, the precise mechanisms of antifungal action in  asthma  remain  unknown. Although  generally  accepted to modulate the immunological response, concerns remain about the azole–corticosteroid interaction. Although  active  against some species of Aspergillus, itraconazole is not active against  all of the fungal species that the human airway is constantly subjected to. Its microbiological activity is further limited by variable absorption and need for monitoring in contrast to newer triazoles that have better oral bioavailability. Further trials in this interesting area are warranted.
41.Thermal bronchoplasty
Targeted treatment of the airway smooth muscle hypertrophy seen in chronic asthma, aside from other changes of airway remodeling  including  goblet  cell  hyperplasia,  increased mucus  secretion,  and  increased  vascularization,  has  been made possible with a novel technique utilizing radiofrequency ablation. Treatment  involves  delivering  thermal  energy through use of a standard bronchoscope into which a catheter containing an expandable basket is inserted. When extended, this comes into circumferential contact with the walls of targeted airways, thus depleting smooth muscle mass with the hope of attenuating the bronchoconstrictor response. In clinical studies, thermal bronchoplasty has been shown to reduce parameters of airways hyperresponsiveness and minimize exacerbations.

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From the book
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- Chiranjeevi Venkatesh
BITS Pilani

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