Page last updated: 2024-10-06

L-carnitine biosynthesis

Proteins (6)

ProteinSynonymsTaxonomy
4-trimethylaminobutyraldehyde dehydrogenaseTMABA-DH; TMABALDH; EC 1.2.1.47; Aldehyde dehydrogenase E3 isozyme; Aldehyde dehydrogenase family 9 member A1; 1.2.1.3; Gamma-aminobutyraldehyde dehydrogenase; 1.2.1.19; R-aminobutyraldehyde dehydrogenaseHomo sapiens (human)
Gamma-butyrobetaine dioxygenaseEC 1.14.11.1; Gamma-butyrobetaine hydroxylase; Gamma-BBH; Gamma-butyrobetaine,2-oxoglutarate dioxygenaseHomo sapiens (human)
Gamma-butyrobetaine dioxygenaseEC 1.14.11.1; Gamma-butyrobetaine hydroxylase; Gamma-BBH; Gamma-butyrobetaine,2-oxoglutarate dioxygenaseRattus norvegicus (Norway rat)
Trimethyllysine dioxygenase, mitochondrial EC 1.14.11.8; Epsilon-trimethyllysine 2-oxoglutarate dioxygenase; TML hydroxylase; TML-alpha-ketoglutarate dioxygenase; TML dioxygenase; TMLDRattus norvegicus (Norway rat)
Trimethyllysine dioxygenase, mitochondrialEC 1.14.11.8; Epsilon-trimethyllysine 2-oxoglutarate dioxygenase; Epsilon-trimethyllysine hydroxylase; TML hydroxylase; TML-alpha-ketoglutarate dioxygenase; TML dioxygenase; TMLDHomo sapiens (human)
4-trimethylaminobutyraldehyde dehydrogenase TMABA-DH; TMABADH; EC 1.2.1.47; Aldehyde dehydrogenase family 9 member A1; 1.2.1.3; Gamma-aminobutyraldehyde dehydrogenase; 1.2.1.19Rattus norvegicus (Norway rat)

Compounds (19)

CompoundDescription
hydronium ion
levocarnitine
Succinate
NADH
CalciumA basic element found in nearly all tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes.
ironA metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN.
ManganeseA trace element with atomic symbol Mn, atomic number 25, and atomic weight 54.94. It is concentrated in cell mitochondria, mostly in the pituitary gland, liver, pancreas, kidney, and bone, influences the synthesis of mucopolysaccharides, stimulates hepatic synthesis of cholesterol and fatty acids, and is a cofactor in many enzymes, including arginase and alkaline phosphatase in the liver. (From AMA Drug Evaluations Annual 1992, p2035)
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
ZincA metallic element of atomic number 30 and atomic weight 65.38. It is a necessary trace element in the diet, forming an essential part of many enzymes, and playing an important role in protein synthesis and in cell division. Zinc deficiency is associated with ANEMIA, short stature, HYPOGONADISM, impaired WOUND HEALING, and geophagia. It is known by the symbol Zn.
MagnesiumA metallic element that has the atomic symbol Mg, atomic number 12, and atomic weight 24.31. It is important for the activity of many enzymes, especially those involved in OXIDATIVE PHOSPHORYLATION.
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
OxygenAn element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.
3-(2,2,2-trimethylhydrazine)propionatestructural analog of gamma-butyrobetaine, also of carnitine; antianginal compound; MET-88 is dihydrate; structure given in first source
gamma-trimethylaminobutyraldehydeintermediate in biosynthesis of carnitine from lysine; structure in first source
nadA coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
alpha-Ketoglutarate
Edetic AcidA chelating agent that sequesters a variety of polyvalent cations such as CALCIUM. It is used in pharmaceutical manufacturing and as a food additive.
trimethyllysinestimulates growth of tumor cells; RN given refers to (S)-isomer
gamma-butyrobetaineRN given refers to hydroxide inner salt