Page last updated: 2024-10-06

Carnitine synthesis

Proteins (4)

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)
Serine hydroxymethyltransferase, cytosolicSHMT; EC 2.1.2.1; Glycine hydroxymethyltransferase; Serine methylaseHomo sapiens (human)
Gamma-butyrobetaine dioxygenaseEC 1.14.11.1; Gamma-butyrobetaine hydroxylase; Gamma-BBH; Gamma-butyrobetaine,2-oxoglutarate dioxygenaseHomo sapiens (human)
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)

Compounds (13)

CompoundDescription
hydronium ion
Succinate
NADH
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.
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
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.
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
carnitineA constituent of STRIATED MUSCLE and LIVER. It is an amino acid derivative and an essential cofactor for fatty acid metabolism.
trimethyllysinestimulates growth of tumor cells; RN given refers to (S)-isomer
gamma-butyrobetaineRN given refers to hydroxide inner salt