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Acylcarnitine 3-Hydroxydodec-3-enedioylcarnitine
smpdb.ca/view/SMP0124024MetabolicThe general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. … and L-carnitine. 3-Hydroxydodec-3-enedioyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA.
Drugs: Biotin · Adenosine phosphate · ATP · Levocarnitine · Magnesium cation · Pyrophosphoric acid +1 moreEnzymes: Long-chain-fatty-acid--CoA ligase 1, Long-chain fatty acid transport protein 13-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
smpdb.ca/view/SMP0000138DiseaseA deficiency in this enzyme results in accumulation of 3-hydroxymethylglutaric acid, 3-hydroxyisovaleric acid, 3-methylcrotonylglycine and 3-methylglutaconic acid (cis and trans form), and methylglutaric … acid in urine; and ammonia in blood.
Enzymes: 3-ketoacyl-CoA thiolase, mitochondrial, 3-hydroxyacyl-CoA dehydrogenase type-2Acylcarnitine 3-[(2R)-2-hydroxy-3-methyl-3-[(phosphonooxy)methyl]butanamido]propanoylcarnitine
smpdb.ca/view/SMP0123534MetabolicThe general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. … As part of this process, 3-[(2R)-2-hydroxy-3-methyl-3-[(phosphonooxy)methyl]butanamido]propanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1).
Drugs: Biotin · Adenosine phosphate · ATP · Levocarnitine · Magnesium cation · Pyrophosphoric acid +1 moreEnzymes: Long-chain-fatty-acid--CoA ligase 1, Long-chain fatty acid transport protein 13-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
smpdb.ca/view/SMP0125660DiseaseA deficiency in this enzyme results in accumulation of 3-hydroxymethylglutaric acid, 3-hydroxyisovaleric acid, 3-methylcrotonylglycine and 3-methylglutaconic acid (cis and trans form), and methylglutaric … acid in urine; and ammonia in blood.
Enzymes: 3-ketoacyl-CoA thiolase, mitochondrial, 3-hydroxyacyl-CoA dehydrogenase type-2Cardiolipin Biosynthesis CL(20:3(11Z,14Z,17Z)/20:3(11Z,14Z,17Z)/20:3(11Z,14Z,17Z)/20:3(11Z,14Z,17Z))
smpdb.ca/view/SMP0248698MetabolicThird, the enzyme 1-acyl-sn-glycerol-3-phosphate acyltransferase converts LPA into phosphatidic acid (PA or 1,2-diacyl-sn-glycerol 3-phosphate) by esterifying an acyl-group to the sn-2 position of the … Second, the mitochondrial outer membrane enzyme glycerol-3-phosphate acyltransferase esterifies an acyl-group to the sn-1 position of sn-glycerol 3-phosphate to form 1-acyl-sn-glycerol 3-phosphate (lysophosphatidic
Drugs: NADH · Cytidine-5'-Triphosphate · sn-glycerol 3-phosphate · Pyrophosphoric acid · Dihydroxyacetone phosphateEnzymes: Phosphatidylglycerophosphatase and protein-tyrosine phosphatase 1, CDP-diacylglycerol--glycerol-3-phosphate 3-phosphatidyltransferase, mitochondrialAcylcarnitine 3-(3-methyl-5-pentylfuran-2-yl)propanoylcarnitine
smpdb.ca/view/SMP0123509MetabolicThe general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. … As part of this process, 3-(3-methyl-5-pentylfuran-2-yl)propanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1).
Drugs: Biotin · Adenosine phosphate · ATP · Levocarnitine · Magnesium cation · Pyrophosphoric acid +1 moreEnzymes: Long-chain-fatty-acid--CoA ligase 1, Long-chain fatty acid transport protein 13-Hydroxyisobutyric Aciduria
smpdb.ca/view/SMP0000522DiseaseSigns and symptoms of 3-hydroxyisobutyric aciduria include developmental delay, dysmorphic facial features, and brain abnormalities. … The excretion of 3-hydroxyisobutyric acid in the urine can range from 170 to 390 mmol/mol of creatinine.
Enzymes: 3-ketoacyl-CoA thiolase, mitochondrial, 3-hydroxyacyl-CoA dehydrogenase type-2Acylcarnitine 3-Butenylcarnitine
smpdb.ca/view/SMP0123321MetabolicThe general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. … As part of this process, 3-Butenic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1).
Drugs: Biotin · Adenosine phosphate · ATP · Levocarnitine · Magnesium cation · Pyrophosphoric acid +1 moreEnzymes: Long-chain-fatty-acid--CoA ligase 1, Long-chain fatty acid transport protein 1Acylcarnitine 3-octenoylcarnitine
smpdb.ca/view/SMP0123369MetabolicThe general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. … As part of this process, 3-octenoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1).
Drugs: Biotin · Adenosine phosphate · ATP · Levocarnitine · Magnesium cation · Pyrophosphoric acid +1 moreEnzymes: Long-chain-fatty-acid--CoA ligase 1, Long-chain fatty acid transport protein 1Acylcarnitine 3-hydroxyoctanedioylcarnitine
smpdb.ca/view/SMP0123405MetabolicThe general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. … As part of this process, 3-hydroxyoctanedioic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1).
Drugs: Biotin · Adenosine phosphate · ATP · Levocarnitine · Magnesium cation · Pyrophosphoric acid +1 moreEnzymes: Long-chain-fatty-acid--CoA ligase 1, Long-chain fatty acid transport protein 1