US2017304355A1PendingUtilityA1

Compositions and methods of use thereof

46
Assignee: SYNEDGEN INCPriority: Sep 11, 2014Filed: Sep 11, 2015Published: Oct 26, 2017
Est. expirySep 11, 2034(~8.2 yrs left)· nominal 20-yr term from priority
A61P 37/08A61P 29/00A61P 31/04A61P 1/04A61P 11/02A61P 11/00A61P 11/08A61K 9/08A61K 47/10A61K 38/14A61K 31/726A61K 45/06A61K 9/0078A61K 31/7036A61K 9/14A61K 9/48A61K 31/427
46
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Claims

Abstract

Described herein are methods for treating or preventing a disease or disorder of the pulmonary system (e.g., cystic fibrosis), respiratory or digestive system in a subject, the methods comprising administering compounds or compositions comprising water soluble polyglucosamine and derivatized polyglucosamine.

Claims

exact text as granted — not AI-modified
1 . A method for treating a subject suffering from a mucosal disease or disorder, comprising administering an effective amount of a poly (acetyl, arginyl) glucosamine (PAAG) comprising the following formula (I): 
       
         
           
           
               
               
           
         
         wherein: 
         n is an integer between 20 and 6000; and 
         each R 1  is independently selected for each occurrence from hydrogen, acetyl, 
       
       
         
           
           
               
               
           
         
         wherein at least 25% of R 1  substituents are H, at least 1% of R 1  substituents are acetyl, and at least 2% of R 1  substituents are 
       
       
         
           
           
               
               
           
         
         wherein the method improves (e.g., enhances, increases) mucociliary transport or clearance, 
         thereby treating a mucosal disease or disorder. 
       
     
     
         2 . The method of  claim 1 , wherein the method reduces the viscosity of mucus. 
     
     
         3 . The method of  claim 1 , wherein the method reduces the elasticity of mucus. 
     
     
         4 . The method of  claim 1 , wherein the method reduces the adhesion of mucus to epithelia (e.g., gastrointestinal or pulmonary epithelia). 
     
     
         5 . The method of  claim 1 , wherein the method reduces the adhesion of bacteria and biofilms to epithelia (e.g., gastrointestinal or pulmonary epithelia). 
     
     
         6 . A method for treating a subject suffering from a gastrointestinal disease or disorder, comprising administering an effective amount of a poly (acetyl, arginyl) glucosamine (PAAG) comprising the following formula (I): 
       
         
           
           
               
               
           
         
         wherein: 
         n is an integer between 20 and 6000; and 
         each R 1  is independently selected for each occurrence from hydrogen, acetyl, 
       
       
         
           
           
               
               
           
         
         wherein at least 25% of R 1  substituents are H, at least 1% of R 1  substituents are acetyl, and at least 2% of R 1  substituents are 
       
       
         
           
           
               
               
           
         
         wherein the method improves (e.g., enhances, increases) mucociliary transport or clearance, 
         thereby treating the gastrointestinal disease or disorder. 
       
     
     
         7 . The method of  claim 6 , wherein the gastrointestinal disease is meconium ileus. 
     
     
         8 . The method of  claim 6 , wherein the gastrointestinal disease is DIOS. 
     
     
         9 . A method for treating a subject suffering from a pulmonary disease or disorder (e.g., improving lung function (e.g., improving the forced expiratory volume in 1 second (FEV 1 ))), comprising administering an effective amount of a poly (acetyl, arginyl) glucosamine (PAAG) comprising the following formula (I): 
       
         
           
           
               
               
           
         
         wherein: 
         n is an integer between 20 and 6000; and 
         each R 1  is independently selected for each occurrence from hydrogen, acetyl, 
       
       
         
           
           
               
               
           
         
         wherein at least 25% of R 1  substituents are H, at least 1% of R 1  substituents are acetyl, and at least 2% of R 1  substituents an 
       
       
         
           
           
               
               
           
         
         wherein the method improves (e.g., enhances, increases) mucociliary transport or clearance, 
         thereby treating the pulmonary disease or disorder. 
       
     
     
         10 . The method of  claim 9 , wherein the method reduces the viscosity of sputum. 
     
     
         11 . The method of  claim 9 , wherein the method reduces the elasticity of sputum. 
     
     
         12 . The method of  claim 9 , wherein the method improves (e.g., enhances, increases) the mobility of sputum. 
     
     
         13 . The method of  claim 9 , wherein the method increases airway surface liquid thickness, increasing fluidity. 
     
     
         14 . The method of  claim 9 , wherein the method improves (e.g., enhances, increases) ciliary beat frequency. 
     
     
         15 . The method of  claim 9 , wherein the method improves resolution of pulmonary exacerbations. 
     
     
         16 . The method of  claim 9 , wherein the method is mucolytic (e.g., removes mucus). 
     
     
         17 . The method of  claim 16 , wherein the PAAG is mucoadhesive. 
     
     
         18 . The method of  claim 17 , wherein the PAAG protects cells (e.g., epithelial cells) from bacterial attachment. 
     
     
         19 . The method of  claim 18 , wherein the method reduces bacterial or biofilm cohesion (e.g., wherein the method reduces biofilm adhesion to the epithelial cell surface). 
     
     
         20 . The method of  claim 9 , wherein the method reduces CF-specific biofilms. 
     
     
         21 . The method of  claim 9 , wherein the method reduces mucus adhesion (e.g., to epithelial cell surfaces). 
     
     
         22 . The method of  claim 9 , wherein the method improves lung function as compared to a subject that has not been treated with the PAAG of formula (I). 
     
     
         23 . The method of  claim 22 , wherein the method improves the forced expiratory volume in 1 second (FEV 1 ). 
     
     
         24 . The method of  claim 9 , wherein the subject has a complication of cystic fibrosis (e.g., lung infection or respiratory congestion) or a symptom thereof. 
     
     
         25 . The method of  claim 24 , wherein the complication of cystic fibrosis is pulmonary exacerbations. 
     
     
         26 . The method of  claim 24 , wherein the complication of cystic fibrosis is a gastrointestinal disease or disorder. 
     
     
         27 . The method of  claim 26 , wherein the gastrointestinal disease is meconium ileus. 
     
     
         28 . The method of  claim 26 , wherein the gastrointestinal disease is DIOS. 
     
     
         29 . The method of  claim 9 , wherein the pulmonary disease or disorder is a chronic disease or disorder. 
     
     
         30 . The method of  claim 29 , wherein the chronic disease is chronic obstructive pulmonary disease (COPD), emphysema, allergic damage, or pulmonary fibrosis. 
     
     
         31 . The method of  claim 9 , wherein the disease is an acute disease. 
     
     
         32 . The method of  claim 31 , wherein the acute disease is inhalation damage (e.g., from smoke, chemicals, or toxins), acute respiratory distress syndrome, or trauma induced respiratory failure. 
     
     
         33 . The method of  claim 9 , further comprising administering an effective amount of an antibacterial agent (e.g., standard of care antibacterial agents to treat infections in CF patients). 
     
     
         34 . The method of  claim 33 , wherein the antibacterial agent is tobramycin, vancomycin, or aztreonam (aztreonam-lysine). 
     
     
         35 . The method of  claim 33 , wherein the method potentiates the efficacy of the antibacterial agent (e.g., antibiotics, e.g., pulmonary antibiotics). 
     
     
         36 . The method of  claim 9 , wherein the administering delivers a composition comprising the PAAG of formula (I). 
     
     
         37 . The method of  claim 36 , wherein the composition is a dry powder composition. 
     
     
         38 . The method of  claim 37 , wherein the composition comprises a vacuum-dried, freeze-dried or spray-dried powder of PAAG. 
     
     
         39 . The method of  claim 37 , wherein the composition is substantially free of impurities. 
     
     
         40 . The method of  claim 36 , wherein the composition is a solution composition (e.g., an aqueous solution composition as described herein, e.g., an aqueous solution composition of neutral osmol). 
     
     
         41 . The method of  claim 36 , wherein the composition is a nebulized composition. 
     
     
         42 . The method of  claim 41 , wherein the nebulized composition comprises PAAG for pulmonary delivery. 
     
     
         43 . The method of  claim 42 , wherein the nebulized composition comprises particles of 1-5 microns in mean particle size diameter. 
     
     
         44 . The method of  claim 9 , wherein the method reduces infection (e.g., bacterial infection). 
     
     
         45 . The method of  claim 44 , wherein the infection is from a bacterial infection (e.g., from a bacteria described herein). 
     
     
         46 . The method of  claim 44 , wherein the bacterial infection is caused by  Pseudomonas aeruginosa.    
     
     
         47 . The method of  claim 44 , wherein the bacterial infection is caused by  Staphylococcus aureus  or methicillin resistant  Staphylococcus aureus.    
     
     
         48 . The method of  claim 44 , wherein the bacterial infection is caused by  Burkholderia  cepacia. 
     
     
         49 . The method of  claim 9 , wherein the method prevents  Burkholderia cepacia  uptake into macrophages. 
     
     
         50 . The method of  claim 9 , wherein the method reduces inflammatory cytokines from pathogenic or damage initiated sources. 
     
     
         51 . The method of  claim 50 , wherein the method reduces inflammation (e.g., pulmonary inflammation). 
     
     
         52 . The method of  claim 51 , wherein the method reduces LPS stimulated TNF-α secretion. 
     
     
         53 . The method of  claim 51 , wherein the method reduces LPS stimulated IL-10 secretion. 
     
     
         54 . The method of  claim 51 , wherein the method reduces LPS stimulated IL-8 secretion. 
     
     
         55 . The method of  claim 51 , wherein the method reduces DNA stimulated IL-8 secretion. 
     
     
         56 . The method of  claim 51 , wherein the method reduces bacterial stimulated IL-8 secretion. 
     
     
         57 . The method of  claim 51 , wherein the method reduces inflammatory cytokine secretion compared to a subject treated with lactoferrin. 
     
     
         58 . The method of  claim 9 , wherein the method reduces pulmonary fibrosis. 
     
     
         59 . The method of  claim 9 , wherein the method increases the accessibility of other therapeutic agents (e.g., anti-bacterials) to bacteria in biofilms. 
     
     
         60 . The method of  claim 9 , wherein the method potentiates the effectiveness of other therapeutic agents (e.g., anti-bacterials) for improving lung function. 
     
     
         61 . The method of  claim 59 , wherein the anti-bacterial agent and PAAG are present at a concentration, or administered at a dose or doses, which result in a bactericidal activity at least 2 logs more effective than the most effective activity in the absence of the PAAG or anti-bacterial agent. 
     
     
         62 . The method of  claim 9 , the method comprising administering a nebulizer solution composition configured for inhaled administration (e.g., a composition as described herein, e.g., a composition comprising PAAG), further comprising a neutral osmol agent (i.e., an agent for achieving neutral osmotic balance). 
     
     
         63 . The method of  claim 62 , wherein the subject is suffering from cystic fibrosis. 
     
     
         64 . The method of  claim 62 , wherein the method provides mucosal clearance in the absence of infection (e.g., relative to a subject that is not treated with the method). 
     
     
         65 . The method of  claim 62 , wherein the composition is administered at about 1 mL to about 3 mL. 
     
     
         66 . The method of  claim 62 , wherein the composition is administered in an amount (e.g, a volume, e.g., nebulized solution volume) sufficient to provide about 0.2 mg to about 3 mg to the subject. 
     
     
         67 . The method of  claim 62 , wherein the composition is administered once daily. 
     
     
         68 . The method of  claim 62 , wherein the composition is administered every other day. 
     
     
         69 . The method of  claim 62 , wherein the composition is administered twice a week. 
     
     
         70 . The method of  claim 62 , wherein the composition is administered once a week. 
     
     
         71 . The method of  claim 62 , further comprising administration of an antibiotic. 
     
     
         72 . The method of  claim 71 , wherein the composition (e.g., a composition as described herein, e.g., a composition comprising PAAG) is administered prior to administration of the antibiotic. 
     
     
         73 . The method of  claim 71 , wherein the composition (e.g., a composition as described herein, e.g., a composition comprising PAAG) is administered concurrently with administration of the antibiotic. 
     
     
         74 . The method of  claim 62 , wherein the average molecular weight of the PAAG is from 20 to 150 kDa. 
     
     
         75 . The method of  claim 74 , wherein the average molecular weight of the PAAG is from 20 to 120 kDa. 
     
     
         76 . The method of  claim 74 , wherein the average molecular weight of the PAAG is from 40 to 100 kDa. 
     
     
         77 . The method of  claim 74 , wherein the average molecular weight of the PAAG is from 70-120 kDa. 
     
     
         78 . The method of  claim 74 , wherein the average molecular weight of the PAAG is from 50-90 kDa. 
     
     
         79 . The method of  claim 62 , wherein the polydispersity index of the PAAG is from 1.0 to 2.5. 
     
     
         80 . The method of  claim 62 , wherein the polydispersity index of the PAAG is from 1.0 to 1.8. 
     
     
         81 . The method of  claim 62 , wherein the pH is about 7 to about 8. 
     
     
         82 . The method of  claim 62 , wherein the PAAG is arginine-functionalized at least 18%. 
     
     
         83 . The method of  claim 82 , wherein the PAAG is arginine-functionalized at between 18% and 30%. 
     
     
         84 . The method of  claim 82 , wherein the PAAG is arginine-functionalized at between 20%-30%. 
     
     
         85 . The method of  claim 82 , wherein the PAAG is greater than 18% arginine-functionalized. 
     
     
         86 . The method of  claim 62 , wherein the neutral osmol agent is a non-fermentable sugar. 
     
     
         87 . The method of  claim 86 , wherein the neutral osmol agent is glycerol, sorbitol, mannitol, xylitol, erythritol or another non-fermentable sugar. 
     
     
         88 . The method of  claim 86 , wherein the non fermenatable sugar is glycerol. 
     
     
         89 . The method of  claim 88 , wherein the glycerol is present in the composition at between 1.2-2.0% v/v. 
     
     
         90 . The method of  claim 88 , wherein the glycerol is present in the composition at between 1.2-1.8% v/v. 
     
     
         91 . The method of  claim 88 , wherein the glycerol is present in the composition at between 1.2-1.6% v/v. 
     
     
         92 . The method of  claim 88 , wherein the glycerol is present in the composition at between 1.2-1.4% v/v. 
     
     
         93 . The method of  claim 88 , wherein the glycerol is present in the composition at between 1.3-1.4% v/v. 
     
     
         94 . The method of  claim 88 , wherein the glycerol is around 1.38% v/v. 
     
     
         95 . The method of  claim 88 , wherein the PAAG is present in the composition at between 0.1-2 mg/ml (i.e., 0.01 to 0.2% w/v). 
     
     
         96 . The method of  claim 88 , wherein the PAAG is present in the composition at between 0.2-1 mg/ml (i.e., 0.02 to 0.1% w/v). 
     
     
         97 . The method of  claim 88 , wherein the PAAG is present in the composition at between 0.2-0.5 mg/ml (i.e., 0.02 to 0.05% w/v). 
     
     
         98 . The method of  claim 88 , wherein the composition comprises a mean particle size diameter of between 1 and 5 microns. 
     
     
         99 . The method of  claim 88 , wherein the osmolality is between 150-550 mOsmol/kg 
     
     
         100 . A dosage form configured for oral administration, comprising: a PAAG comprising the following formula (I): 
       
         
           
           
               
               
           
         
         wherein: 
         n is an integer between 20 and 6000; and 
         each R 1  is independently selected for each occurrence from hydrogen, acetyl, 
       
       
         
           
           
               
               
           
         
         wherein at least 25% of R 1  substituents are H, at least 1% of R 1  substituents are acetyl, and at least 2% of R 1  substituents are 
       
       
         
           
           
               
               
           
         
         further comprising a neutral osmol agent (i.e., an agent for achieving neutral osmotic balance). 
       
     
     
         101 . The dosage form of  claim 100 , wherein the dosage form is a capsule or gel-capsule. 
     
     
         102 . A nebulizer solution composition configured for inhaled administration, comprising: a PAAG comprising the following formula (I): 
       
         
           
           
               
               
           
         
         wherein: 
         n is an integer between 20 and 6000; and 
         each R 1  is independently selected for each occurrence from hydrogen, acetyl, 
       
       
         
           
           
               
               
           
         
         wherein at least 25% of R 1  substituents are H, at least 1% of R 1  substituents are acetyl, and at least 2% of R 1  substituents are 
       
       
         
           
           
               
               
           
         
         further comprising a neutral osmol agent (i.e., an agent for achieving neutral osmotic balance). 
       
     
     
         103 . The composition of  claim 102 , wherein the average molecular weight of the PAAG is from 20 to 150 kDa. 
     
     
         104 . The composition of  claim 103 , wherein the average molecular weight of the PAAG is from 20 to 120 kDa. 
     
     
         105 . The composition of  claim 103 , wherein the average molecular weight of the PAAG is from 40 to 100 kDa 
     
     
         106 . The composition of  claim 103 , wherein the average molecular weight of the PAAG is from 70-120 kDa 
     
     
         107 . The composition of  claim 103 , wherein the average molecular weight of the PAAG is from 50-90 kDa 
     
     
         108 . The composition of  claim 102 , wherein the polydispersity index of the PAAG is from 1.0 to 2.5. 
     
     
         109 . The composition of  claim 102 , wherein the polydispersity index of the PAAG is from 1.0 to 1.8. 
     
     
         110 . The composition of  claim 102 , wherein the pH is about 7 to about 8. 
     
     
         111 . The composition of  claim 102 , wherein the PAAG is arginine-functionalized at least 18%. 
     
     
         112 . The composition of  claim 111 , wherein the PAAG is arginine-functionalized at between 18% and 30%. 
     
     
         113 . The composition of  claim 111 , wherein the PAAG is arginine-functionalized at between 20%-30%. 
     
     
         114 . The composition of  claim 111 , wherein the PAAG is greater than 18% arginine-functionalized. 
     
     
         115 . The composition of  claim 102 , wherein the neutral osmol agent is a non-fermentable sugar. 
     
     
         116 . The composition of  claim 115 , wherein the neutral osmol agent is glycerol, sorbitol, mannitol, xylitol, erythritol or another non-fermentable sugar. 
     
     
         117 . The composition of  claim 115 , wherein the non fermenatable sugar is glycerol. 
     
     
         118 . The composition of  claim 115 , wherein the glycerol is present in the composition at between 1.2-2.0% v/v. 
     
     
         119 . The composition of  claim 115 , wherein the glycerol is present in the composition at between 1.2-1.8% v/v. 
     
     
         120 . The composition of  claim 115 , wherein the glycerol is present in the composition at between 1.2-1.6% v/v. 
     
     
         121 . The composition of  claim 115 , wherein the glycerol is present in the composition at between 1.2-1.4% v/v. 
     
     
         122 . The composition of  claim 115 , wherein the glycerol is present in the composition at between 1.3-1.4% v/v. 
     
     
         123 . The composition of  claim 115 , wherein the glycerol is around 1.38% v/v. 
     
     
         124 . The composition of  claim 115 , wherein the PAAG is present in the composition at between 0.1-2 mg/ml (i.e., 0.01 to 0.2% w/v). 
     
     
         125 . The composition of  claim 115 , wherein the PAAG is present in the composition at between 0.2-1 mg/ml (i.e., 0.02 to 0.1% w/v). 
     
     
         126 . The composition of  claim 115 , wherein the PAAG is present in the composition at between 0.2-0.5 mg/ml (i.e., 0.02 to 0.05% w/v). 
     
     
         127 . The composition of  claim 115 , wherein the composition comprises a mean particle size diameter of between 1 and 5 microns. 
     
     
         128 . The composition of  claim 115 , wherein the osmolality is between 150-550 mOsmol/kg

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