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Nutritional supplement or pharmaceutical preparation comprising triglycerides with seven-carbon fatty acid

A technology of fatty acid and fatty acid metabolism, applied in medical preparations containing active ingredients, drug combinations, anhydride/acid/halide active ingredients, etc.

Inactive Publication Date: 2008-08-06
BAYLOR RES INST
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  • Summary
  • Abstract
  • Description
  • Claims
  • Application Information

AI Technical Summary

Problems solved by technology

However, so far there is no indication that human consumption of seven-carbon fatty acids is safe or provides any particular nutritional benefit to humans

Method used

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  • Nutritional supplement or pharmaceutical preparation comprising triglycerides with seven-carbon fatty acid
  • Nutritional supplement or pharmaceutical preparation comprising triglycerides with seven-carbon fatty acid
  • Nutritional supplement or pharmaceutical preparation comprising triglycerides with seven-carbon fatty acid

Examples

Experimental program
Comparison scheme
Effect test

Embodiment 1

[0134] Example 1: Supplementation in Cell Lines

[0135] Addition of n-heptanoic acid to a cultured cell line (fibroblasts) from a lethal translocase-deficient patient showed a successful oxidation reaction.

[0136] Amnion cells from a sibling who died of severe translocase deficiency at 4 days of age were obtained to test their survival conditions in fatty acid metabolism. These experiments revealed that the fetus was also severely deficient in translocase.

[0137] Fibroblasts from dead siblings and amniocytes from the fetus were evaluated for n-heptanoic acid (C 7 ) fatty acid metabolism (Yang et al., 1998, "Identification of four novel mutants in patients deficient in carnitine palmitoyltransferase II (CPT II), Mol Genet Metab 64: 229-236). The mass spectrometry results are given as follows: palmitate in Figure 3A and enanthin triglyceride in Figure 3B, where fibroblasts were obtained from dead siblings, and palmitate in Figure 4A, in Figure 3B Figure 4B is enanthin tr...

Embodiment 2

[0144] Example 2: In Vivo Utilization of Enanthate Triglyceride Supplementation in Patients with Severe Translocase Deficiency

[0145] Infants with severe neonatal translocase deficiency identified in Example 1 were successfully treated with the low-fat formulation supplemented with enanthin triglycerides. Furthermore, the relationship between clinical response to enanthate triglyceride treatment and in vitro mass spectrometry analysis of infant amnion cells was mutually supported.

[0146] At 38 weeks' gestation, infants whose amniocytes tested positive for severe translocase deficiency as described in Example 1 were delivered. Cord blood total and free carnitine levels, as well as individual acylcarnitine levels, were analyzed by tandem mass spectrometry (Yang et al., 1998, "Identification of four novel mutants in carnitine palmitoyltransferase II (CPTII) deficient patients" , Mol Genet Metab 64:229-236). The levels of these substances in the mother's blood are also teste...

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Abstract

A seven-carbon fatty acid, preferably n-heptanoic acid, has been identified as an excellent energy source for patients suffering from inherited metabolic disorders or acquired metabolic derangements, especially defects in long-chain fatty acid metabolism. A seven-carbon fatty acid can also be provided in a nutritional supplement for patients who need an increase in the energy production derived from fatty acid metabolism.

Description

[0001] invention technical field [0002] The present invention relates to nutritional or dietary compositions or supplements. Background of the invention [0003] Fatty acid oxidation plays an important role in energy production and is required during fasting. Severe disturbances in fatty acid metabolism may cause problems ranging from skeletal and / or myocardial weakness to episodes of metabolic asphyxia and death (similar to sudden infant death syndrome). These disorders manifest as severe cardiomyopathy, hypoglycemia, myopathy, microcystic fatty deposits in affected organs, and / or fulminant hepatic failure. Patients born with inherited disorders of fatty acid metabolism are often near death or recurrently severely debilitated by the inability to produce energy from fatty acid metabolism. Premature infants need to maintain high levels of blood sugar, their daily diet often does not provide sufficient carbohydrate energy sources, and their fat metabolism enzymes are insuffi...

Claims

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Application Information

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IPC IPC(8): A61K31/23A61K31/20A61P3/00A23L1/30A61K31/19A61K31/215A61P3/02
CPCA23L1/3008A23L33/12A61P3/00A61P3/02A61P3/08A61P43/00A61P5/00A61P9/00A61K31/20
Inventor C·R·雷
Owner BAYLOR RES INST
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