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Matched Description: are phosphatidylethanolamine, phosphatidylglycerol, and cardiolipin. All phospholipids contain sn … -glycerol-3-phosphate esterified with fatty acids at the sn-1 and sn-2 positions. The reaction starts … phosphatidylglycerophosphatase. These 2 products combine to produce a cardiolipin and an ethanolamine. The L-1 … phosphatidyl-glycerol can also interact with cardiolipin synthase resulting in a glycerol and a cardiolipin.

Matched Protein Synonyms: Zrt- and Irt-like protein 8

Matched Compound Synonyms: [p(oh)3se] … [po(oh)3] … Ammonia (conc 20% or greater) … [po(oh)3] … [seo2(oh)2]

Matched Description: include hypothermia, dystonia and irritability right before an episode. They also include loss of … consciousness, convulsions, and muscle rigidity. Rarely does this condition manifest between 1 to 3 years

Matched Protein Synonyms: Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase … Dehydrogenase E1 and transketolase domain-containing protein 1

Matched Compound Synonyms: H-lys-oh … 3-oh-butyryl-coa … 3-oh-butyryl-coenzyme a … H-lys-oh … H-lys-oh

Matched Description: Saccharopinuria (also known as: saccharopinemia, saccharopine dehydrogenase deficiency, and alpha … disease characterized by high concentrations of saccharopine in the plasma and urine.It is caused by the … ketoglutarate reductase (LKR) domain which catalyzes the conversion of lysine to saccharopine, and a

Matched Protein Synonyms: Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase … Dehydrogenase E1 and transketolase domain-containing protein 1

Matched Compound Synonyms: H-lys-oh … 3-oh-butyryl-coa … 3-oh-butyryl-coenzyme a … H-lys-oh … H-lys-oh

Matched Description: Saccharopinuria (also known as: saccharopinemia, saccharopine dehydrogenase deficiency, and alpha … semialdehyde synthase (AASS) enzyme and causes an increase in saccharopine in the urine. Saccharopinuria … is another form of hyperlysinemia. AASS has lysine ketoglutarate reductase (LKR) and saccharopine … enzyme results in accumulation of citrulline, lysine and saccharopin in the plasma; lysine in the … spinal fluid; and citrulline, lysine and saccharopine in the urine. Symptoms include growth and mental retardation.

Matched Protein Synonyms: Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase … Dehydrogenase E1 and transketolase domain-containing protein 1

Matched Compound Synonyms: H-lys-oh … 3-oh-butyryl-coa … 3-oh-butyryl-coenzyme a … H-lys-oh … H-lys-oh

Matched Description: include hypothermia, dystonia and irritability right before an episode. They also include loss of … consciousness, convulsions, and muscle rigidity. Rarely does this condition manifest between 1 to 3 years

Matched Protein Synonyms: Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase … Dehydrogenase E1 and transketolase domain-containing protein 1

Matched Compound Synonyms: H-lys-oh … 3-oh-butyryl-coa … 3-oh-butyryl-coenzyme a … [oh(oh)] … H-lys-oh

Matched Description: , avocados, bananas, milk, cheese, yogurt, cottage cheese, lima beans, pumpkin seeds, and sesame seeds … (noradrenaline), and epinephrine (adrenaline). Depicted in this pathway is the conversion of … ), the incorporation of phenylalanine and/or tyrosine into polypeptides (via tyrosyl tRNA synthetase … and phenylalyl tRNA synthetase) and the conversion of phenylalanine to tyrosine via phenylalanine … which the body disposes of excess phenylalanine, and as a source of the amino acid tyrosine. The

Matched Compound Synonyms: 2-oxo-3-phenylpropanoic acid (mixture oxo and keto) … Ammonia (conc 20% or greater) … [oh(oh)] … Ammonia (conc 20% or greater) … [oh(oh)]

Matched Description: Phenylalanine and tyrosine are two of the 22 total amino acids, 21 of which are used in humans … , and can only be obtained from food, or by recycling other similar molecules. This pathway begins with … oxygen molecule, forming phenylpyruvic acid. Phenylpyruvic acid can then be transformed to and from L

Matched Compound Synonyms: 2-oxo-3-phenylpropanoic acid (mixture oxo and keto) … Ammonia (conc 20% or greater) … [oh(oh)] … Ammonia (conc 20% or greater) … [oh(oh)]

Matched Description: -ketoglutarate reductase and saccharopine dehydrogenase. In hyperlysinemia type I, both enzymatic … then cleaved to alpha-aminoadipic semialdehyde and glutamic acid by saccharopine dehydrogenase … of most proteins. Pipecolic acid can also be increased in serum and urine, while ornithine is … vomiting, and diarrhea. For the vast majority of people, hyperlysinemia typically causes no health … problems, and most people with elevated lysine levels are unaware that they have this condition.

Matched Protein Synonyms: Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase … Dehydrogenase E1 and transketolase domain-containing protein 1

Matched Compound Synonyms: H-lys-oh … 3-oh-butyryl-coa … 3-oh-butyryl-coenzyme a … H-lys-oh … H-lys-oh

Matched Description: -hydroxybutyric acid, 3-hydroxyglutaric acid, glutaconic acid, glutaric acid, and ketone bodies in … urine. Symptoms include encephalopathy, grimacing, dystonia, metabolic acidosis, and hygroma. Treatment … includes a low-protein diet, L-carnitine, riboflavin, and anticonvulsants.

Matched Protein Synonyms: Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase … Dehydrogenase E1 and transketolase domain-containing protein 1

Matched Compound Synonyms: H-lys-oh … 3-oh-butyryl-coa … 3-oh-butyryl-coenzyme a … H-lys-oh … H-lys-oh

Matched Description: -ketoglutarate reductase and saccharopine dehydrogenase. In hyperlysinemia type I, both enzymatic functions … then cleaved to alpha-aminoadipic semialdehyde and glutamic acid by saccharopine dehydrogenase … of most proteins. Pipecolic acid can also be increased in serum and urine, while ornithine is … vomiting, and diarrhea. For the vast majority of people, hyperlysinemia typically causes no health … problems, and most people with elevated lysine levels are unaware that they have this condition.

Matched Protein Synonyms: Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase … Dehydrogenase E1 and transketolase domain-containing protein 1

Matched Compound Synonyms: H-lys-oh … 3-oh-butyryl-coa … 3-oh-butyryl-coenzyme a … [oh(oh)] … H-lys-oh