US2005027006A1PendingUtilityA1

Methods and materials for treating conditions associated with metabolic disorders

51
Priority: Jul 28, 2003Filed: Apr 17, 2004Published: Feb 3, 2005
Est. expiryJul 28, 2023(expired)· nominal 20-yr term from priority
Inventors:Reuben Matalon
A61P 3/00A61P 3/02A61K 31/197A61K 47/183A61K 31/198A61K 31/195
51
PatentIndex Score
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Claims

Abstract

Disclosed are methods for treating a subject suffering from phenylketonuria and/or phenylalanemia. The methods include, in part, enterally administering to the subject a LNAA supplement in which the weight ratio of Leu to Val is greater than 2:1; in which the weight ratio of Leu to iLeu is greater than 3:1; or which includes one or more LNAAs and which further includes Lys. LNAA supplements are also disclosed. Also disclosed are methods for treating a subject suffering from a condition involving a metabolic disorder involving the metabolism of a first amino acid X. The method includes enterally administering to the subject a composition which (i) is substantially free from the first amino acid X and (ii) which includes a second amino acid Y that competes with amino acid X at a gastrointestinal tract transporter.

Claims

exact text as granted — not AI-modified
1 . A method for treating a subject suffering from phenylketonuria and/or phenylalanemia, said method comprising: 
 enterally administering to the subject a LNAA supplement in which the weight ratio of Leu to Val is greater than 2:1.    
     
     
         2 . A method according to  claim 1 , wherein the LNAA supplement is substantially free from phenylalanine.  
     
     
         3 . A method for treating a subject suffering from phenylketonuria and/or phenylalanemia, said method comprising: 
 enterally administering to the subject a LNAA supplement in which the weight ratio of Leu to iLeu is greater than 3:1.    
     
     
         4 . A method according to  claim 3 , wherein the weight ratio of Leu to Val in the LNAA supplement is greater than 2:1.  
     
     
         5 . A method according to  claim 4 , wherein the LNAA supplement is substantially free from phenylalanine.  
     
     
         6 . A method for treating a subject suffering from phenylketonuria and/or phenylalanemia, said method comprising: 
 enterally administering to the subject a LNAA supplement which comprises one or more LNAAs and which further comprises Lys.    
     
     
         7 . A method according to  claim 6 , wherein the LNAA supplement comprises Leu.  
     
     
         8 . A method according to  claim 6 , wherein the LNAA supplement comprises Leu and wherein the weight ratio of Leu to iLeu in the LNAA supplement is greater than about 0.5:1.  
     
     
         9 . A method according to  claim 6 , wherein the LNAA supplement comprises Leu and wherein the weight ratio of Leu to iLeu in the LNAA supplement is greater than 3:1.  
     
     
         10 . A method according to  claim 6 , wherein the LNAA supplement comprises Leu and wherein the weight ratio of Leu to Val in the LNAA supplement is greater than about 0.5:1.  
     
     
         11 . A method according to  claim 6 , wherein the LNAA supplement comprises Leu and wherein the weight ratio of Leu to Val in the LNAA supplement is greater than 2:1.  
     
     
         12 . A method according to  claim 6 , wherein the LNAA supplement comprises Leu; wherein the weight ratio of Leu to iLeu in the LNAA supplement is greater than about 0.5:1; and wherein the weight ratio of Leu to Val in the LNAA supplement is greater than about 0.5:1.  
     
     
         13 . A method according to  claim 12 , wherein the weight ratio of Leu to iLeu in the LNAA supplement is greater than 3:1.  
     
     
         14 . A method according to  claim 12 , wherein the weight ratio of Leu to Val in the LNAA supplement is greater than 2:1.  
     
     
         15 . A method according to  claim 6 , wherein the LNAA supplement comprises Leu; wherein the weight ratio of Leu to iLeu in the LNAA supplement is greater than 3:1; and wherein the weight ratio of Leu to Val in the LNAA supplement is greater than 2:1.  
     
     
         16 . A method according to  claim 6 , wherein the LNAA supplement is substantially free from phenylalanine.  
     
     
         17 . A LNAA supplement comprising Leu and Val in which the weight ratio of Leu to Val is greater than 2:1.  
     
     
         18 . A LNAA supplement according to  claim 17 , wherein the LNAA supplement is substantially free from phenylalanine.  
     
     
         19 . A LNAA supplement comprising Leu and iLeu in which the weight ratio of Leu to iLeu is greater than 3:1.  
     
     
         20 . A LNAA supplement according to  claim 19 , wherein the weight ratio of Leu to Val in the LNAA supplement is greater than 2:1.  
     
     
         21 . A LNAA supplement according to  claim 20 , wherein the LNAA supplement is substantially free from phenylalanine.  
     
     
         22 . A LNAA supplement comprising one or more LNAAs and further comprising Lys.  
     
     
         23 . A LNAA supplement according to  claim 22 , wherein the LNAA supplement comprises Leu.  
     
     
         24 . A LNAA supplement according to  claim 22 , wherein the LNAA supplement comprises Leu and wherein the weight ratio of Leu to iLeu in the LNAA supplement is greater than about 0.5:1.  
     
     
         25 . A LNAA supplement according to  claim 22 , wherein the LNAA supplement comprises Leu and wherein the weight ratio of Leu to iLeu in the LNAA supplement is greater than 3:1.  
     
     
         26 . A LNAA supplement according to  claim 22 , wherein the LNAA supplement comprises Leu and wherein the weight ratio of Leu to Val in the LNAA-supplement is greater than about 0.5:1.  
     
     
         27 . A LNAA supplement according to  claim 22 , wherein the LNAA supplement comprises Leu and wherein the weight ratio of Leu to Val in the LNAA supplement is greater than 2:1.  
     
     
         28 . A LNAA supplement according to  claim 22 , wherein the LNAA supplement comprises Leu; wherein the weight ratio of Leu to iLeu in the LNAA supplement is greater than about 0.5:1; and wherein the weight ratio of Leu to Val in the LNAA supplement is greater than about 0.5:1.  
     
     
         29 . A LNAA supplement according to  claim 28 , wherein the weight ratio of Leu to iLeu in the LNAA supplement is greater than 3:1.  
     
     
         30 . A LNAA supplement according to  claim 28 , wherein the weight ratio of Leu to Val in the LNAA supplement is greater than 2:1.  
     
     
         31 . A LNAA supplement according to  claim 22 , wherein the LNAA supplement comprises Leu; wherein the weight ratio of Leu to iLeu in the LNAA supplement is greater than 3:1; and wherein the weight ratio of Leu to Val in the LNAA supplement is greater than 2:1.  
     
     
         32 . A LNAA supplement according to  claim 22 , wherein the LNAA supplement is substantially free from phenylalanine.  
     
     
         33 . A LNAA supplement according to  claim 22 , wherein the LNAA supplement comprises, per 500 mg of LNAA supplement: 
 from about 100 mg to about 290 mg of Tyr;    from about 25 mg to about 75 mg of Trp;    from about 15 mg to about 50 mg of Met;    from about 15 mg to about 55 mg of iLeu;    from about 15 mg to about 50 mg of Threo;    from about 15 mg to about 55 mg of Val;    from about 15 mg to about 200 mg of Leu;    from about 10 mg to about 30 mg of His; and    from about 5 mg to about 200 mg of Lys.    
     
     
         34 . A LNAA supplement according to  claim 33 , wherein the LNAA supplement comprises, per 500 mg of LNAA supplement, from about 10 mg to about 30 mg of Lys.  
     
     
         35 . A LNAA supplement according to  claim 33 , wherein the LNAA supplement is substantially free from arginine.  
     
     
         36 . A LNAA supplement according to  claim 33 , wherein the LNAA supplement is substantially free from phenylalanine.  
     
     
         37 . A LNAA supplement comprising, per 600 mg of LNAA supplement: 
 from about 100 mg to about 290 mg of Tyr;    from about 30 mg to about 90 mg of Trp;    from about 25 mg to about 75 mg of Met;    from about 15 mg to about 45 mg of iLeu;    from about 15 mg to about 50 mg of Threo;    from about 15 mg to about 50 mg of Val;    from about 40 mg to about 200 mg of Leu;    from about 15 mg to about 45 mg of His; and    from about 15 mg to about 50 mg of Arg.    
     
     
         38 . A LNAA supplement according to  claim 37 , wherein the LNAA supplement further comprises Lys.  
     
     
         39 . A LNAA supplement according to  claim 37 , wherein the LNAA supplement further comprises, per 600 mg of LNAA supplement, from about 5 mg to about 200 mg of Lys.  
     
     
         40 . A LNAA supplement according to  claim 37 , wherein the LNAA supplement is substantially free from phenylalanine.  
     
     
         41 . A method for treating a subject suffering from a condition involving a metabolic disorder involving the metabolism of a first amino acid X, said method comprising: 
 enterally administering to the subject a composition which is substantially free from said first amino acid X and which comprises a second amino acid Y that competes with amino acid X at a gastrointestinal tract transporter.    
     
     
         42 . A method according to  claim 41 , wherein the condition is not phenylketonuria and/or phenylalanemia.  
     
     
         43 . A method according to  claim 42 , wherein the gastrointestinal tract transporter is a Caco-2 cell transporter.  
     
     
         44 . A method according to  claim 41 , wherein the gastrointestinal tract transporter is a Caco-2 cell transporter.  
     
     
         45 . A method according to  claim 44 , wherein the condition is tyrosinemia; wherein the first amino acid X is tyrosine; and wherein the second amino acid Y is selected from Phe, Leu, Trp, Lys, His, and combinations thereof.  
     
     
         46 . A method according to  claim 44 , wherein the condition is tyrosinemia; wherein the first amino acid X is selected from phenylalanine, tyrosine, and combinations thereof; and wherein the second amino acid Y is selected from Leu, Trp, Lys, His, and combinations thereof.  
     
     
         47 . A method according to  claim 44 , wherein the condition is alkaptonuria; wherein the first amino acid X is selected from phenylalanine, tyrosine, and combinations thereof; and wherein the second amino acid Y is selected from Leu, Trp, Lys, His, and combinations thereof.  
     
     
         48 . A method according to  claim 44 , wherein the condition is homocystinuria; wherein the first amino acid X is methionine; and wherein the second amino acid Y is an amino acid that competes with methionine at a gastrointestinal tract transporter.  
     
     
         49 . A method according to  claim 44 , wherein the condition is a disorder affecting metabolism of a branched amino acid selected from leucine, isoleucine, valine, and combinations thereof; wherein the first amino acid X is selected from leucine, isoleucine, valine, and combinations thereof; and wherein the second amino acid Y is an amino acid that competes with the first amino acid X at a gastrointestinal tract transporter.  
     
     
         50 . A method according to  claim 49 , wherein the condition is selected from maple syrup urine disease, isovaleric acidemia, methylmalonic acidemia, and propionic acidemia.  
     
     
         51 . A method according to  claim 41 , wherein said method further comprises: 
 restricting the subject's dietary intake of the first amino acid X.    
     
     
         52 . A method according to  claim 41 , wherein said method further comprises: 
 not restricting the subject's dietary intake of the first amino acid X.    
     
     
         53 . A method according to  claim 52 , wherein said enteral administration is carried out substantially at mealtime.  
     
     
         54 . A method according to  claim 41 , wherein said enteral administration is carried out substantially at mealtime.  
     
     
         55 . A method according to  claim 41 , wherein said enteral administration is carried out orally.

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