15 results about "Maple syrup" patented technology

1. The name of the product of this design: Birkenstocks (maple syrup muffins). 2. Purpose of the product of this design: to be worn on the feet. 3. The design key point of this appearance design product lies in the combination of shape and pattern. 4. The picture or photo that best illustrates the design points: three-dimensional picture.

The present disclosure relates to a sugar substitute composition comprising components selected from at least one regular sugar, maple syrup, and honey, at least one herb extract, at least one soluble fiber, at least one natural sweetener, at least one sugar alcohol, at least one anti-caking agent, and at least one vitamin and minerals. The composition substantially reduces the ill-effects of consuming regular sugar.

The present disclosure provides alcoholic drinks using maple syrup as a raw material and methods for the preparation. The maple syrup, with its sugar concentration of 66% and its pristine and pure properties, stimulates an easy initiation of a fermentation, and the methods do not yield any waste or impurities after fermentation, which is more environmentally friendly. Additionally, the present disclosure employs a maple wood barrel storing and aging the alcoholic drink, augmenting the aroma and quality of the maple syrup wine further.

An edible organic baby teether is disclosed, comprising a frozen therapeutic confectionery formulated from organic nut butter and natural sweeteners, shaped to be safely chewed and consumed by infants for relief of teething discomfort. The invention provides a dual-function teething aid that offers both cooling therapy and nutritional benefit, eliminating the risks associated with synthetic or non-edible teethers. In the preferred embodiment, the composition includes almond, peanut, or cashew butter blended with organic honey, agave nectar, or maple syrup, molded into a popsicle form with an integrated edible or biodegradable handle for infant grasping. When frozen, the product delivers a soothing, gum-numbing effect, and as it softens, it may be safely ingested, providing essential nutrients such as proteins and healthy fats. A method of manufacture is also disclosed, including steps of mixing, molding, freezing, and packaging the organic composition to achieve a soft-freeze texture optimized for infant safety and enjoyment.

The present invention features palatable pharmaceutical compositions including sodium phenylbutyrate and methods for the treatment of inborn errors of metabolism (e.g., Maple Syrup Urine Disease or Urea Cycle Disorders), neurodegenerative disorders such as Parkinson's disease, spinal muscular atrophy, dystonia, or inclusion-body myositis with such compositions.

This disclosure relates to mRNA therapy for the treatment of maple syrup urine disease (MSUD). mRNAs for use in the invention, when administered in vivo, encode branched chain α-ketoacid dehydrogenase complex (BCKDC) E1α, E1β, or E2mRNA therapies of the disclosure increase and / or restore deficient levels of E1α, E1β, or E2 expression and / or BCKDC activity in subjects. mRNA therapies of the invention further decrease abnormal accumulation of branched chain amino acids associated with deficient BCKDC activity in subjects.

1. The name of the design product: wine bottle (maple tree cellar). 2. The use of the design product: as a package for wine. 3. The design points of the design product: the shape, pattern and combination of the product of the patent design. 4. The picture or photo that best indicates the design points: perspective view.

1. Name of the Design Product: Food Packaging Box (Maple Syrup Flavored Biscuits). 2. Use of the Design Product: Packaging food. 3. Design Key Points of the Design Product: The combination of shape and pattern. 4. Picture or Photograph that Best Illustrates the Design Key Points: Perspective view.

The present invention relates generally to isolated mitochondria derived from mtDNA-depleted cells (RhoO-mito) that are advantageously used as delivery vehicles for heterogenous proteins or nucleic acids or chemical agents. In some embodiments, proteins or nucleic acids or chemical agents are introduced into a cell, transported into, transferred or expressed in the mitochondria, thereafter or before mtDNA is removed and mtDNA-free mitochondria are extracted from the cell. These mitochondria are used for therapeutic treatment of mitochondria-related diseases such as maple syrup syndrome, citrin deficiency, Nieman-Pick disease, X-linked adrenoleukodystrophy (X-ALD), Pyruvate Dehydrogenase Deficiency, respiratory chain diseases, mutations in mitochondrial respiratory chain proteins, fatty acid metabolism disorders, or cancer with reduced likelihood of causing an immunogenic response. Additionally, in some embodiments the present invention relates to isolated mitochondria, including but not necessarily isolated mitochondria derived from mtDNA-depleted cells (RhoO-mito), that are exogenously introduced into cells to enhance production of endogenous or heterogenous proteins.

The present invention belongs to the technical field of food, and discloses a Brazil nut ice cream and a preparation technology thereof, the ice cream comprises the following preparation raw materials: Brazil nuts, Canada maple sugar, coconut juice, milk and auxiliary materials, and the auxiliary materials comprise coconut oil, single cream, butter and a sweetening agent. The Brazil nuts, the Canada maple sugar, the coconut juice and the milk are used as main raw materials, baking treatment is performed on the Brazil nuts according to the specific properties of the Brazil nuts, the honey processing technology of the Canada maple sugar and the coconut juice on the Brazil nuts is optimized, and the influence of direct addition of the Brazil nuts on the taste of the ice cream is reduced; the content of sodium ions in the ice cream can be reduced, the content of selenium can be increased, and the ice cream which is good in flavor and taste and rich in nutrition can be prepared.

To provide a pharmaceutical product that has B0AT1 inhibitory activity. [Solution] The present invention is based on formula (I): [C1] TIFF2026066243000332.tif44170 [In the formula, each symbol is as described in the specification.] The present invention relates to a pharmaceutical composition containing a compound represented by or a salt thereof, and a B0AT1 inhibitor. One embodiment of the present invention is a pharmaceutical suitable for the prevention and / or treatment of amino acid metabolic disorders such as phenylketonuria, urea cycle disorders, hypertyrosinemia (types 1-3), hypermethioninemia, maple syrup urine disease, homocystinuria, nonketotic hyperglycinemia, propionic acidemia, methylmalonic acidemia, and isovaleric acidemia.

To provide a palatable sodium phenylbutyrate formulation for improving patient compliance, methods of making the same, and methods of treatment.SOLUTION: Palatable compositions comprising sodium phenylbutyrate and a taste-mask coating, and methods of treating congenital metabolic disorder (e.g., Maple syrup urine disease or urea cycle disorders), neurodegenerative disorders such as Parkinson's, spinal muscular atrophy, dystonia, or inclusion body myositis using such compositions are provided.SELECTED DRAWING: None

A system for non-invasive microwave testing a bottle of liquid may include an emission probe for emitting a microwave signal through a wall of the bottle into the liquid and a detection probe for receiving at least a portion of the microwave signal from the liquid via the wall. The system includes a classifier configured to distinguish olive oil from some other common vegetable oils, to distinguish real from some imitation maple syrups, and to distinguish between several alcoholic beverages.

In some aspects the disclosure provides compositions and methods for promoting expression of functional BCKDHA protein, which is the E1-alpha subunit of the branched-chain alpha-keto acid (BCAA) dehydrogenase complex, in a subject. In some aspects the disclosure provides compositions and methods for promoting expression of functional BCKDHB protein, which is the E1-beta subunit of the branched-chain alpha-keto acid (BCAA) dehydrogenase complex, in a subject. In some aspects the disclosure provides compositions and methods for promoting expression of functional BCKDHA and BCKDHB proteins, in a subject. In some embodiments, the disclosure provides methods of treating a subject having Maple Syrup Urine Disease (MSUD).

The present invention relates to a compound having a BOAT1 inhibitory action and represented by the following formula (I):wherein each symbol is as defined in the specification, or a salt thereof. In addition, the present invention relates to a BOAT1 inhibitor containing the aforementioned compound and a pharmaceutical composition containing the aforementioned compound. One embodiment of the present invention is, for example, a medicament preferable for the prophylaxis and / or treatment of amino acid metabolism disorders such as phenylketonuria, urea cycle disorder, hypertyrosinemia (types 1-3), hypermethioninemia, maple syrup urine disease, homocystinuria, nonketotic hyperglycinemia, propionic acidemia, methylmalonic acidemia, isovaleric acidemia and the like.