Trimethyllysine dioxygenase

Last updated
trimethyllysine dioxygenase
Identifiers
EC no. 1.14.11.8
CAS no. 74622-49-4
Databases
IntEnz IntEnz view
BRENDA BRENDA entry
ExPASy NiceZyme view
KEGG KEGG entry
MetaCyc metabolic pathway
PRIAM profile
PDB structures RCSB PDB PDBe PDBsum
Gene Ontology AmiGO / QuickGO
Search
PMC articles
PubMed articles
NCBI proteins

In enzymology, a trimethyllysine dioxygenase (TMLH; EC 1.14.11.8) is an enzyme that catalyzes the chemical reaction

N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2

TMLH is a member of the alpha-ketoglutarate-dependent hydroxylases superfamily. The 3 substrates of this enzyme are N6,N6,N6-trimethyl-L-lysine, 2-oxoglutarate, and O2, whereas its 3 products are 3-hydroxy-N6,N6,N6-trimethyl-L-lysine, succinate, and CO2.

This enzyme belongs to the family of oxidoreductases, specifically those acting on paired donors, with O2 as oxidant and incorporation or reduction of oxygen. The oxygen incorporated need not be derived from O2 with 2-oxoglutarate as one donor, and incorporation of one atom o oxygen into each donor. The systematic name of this enzyme class is N6,N6,N6-trimethyl-L-lysine,2-oxoglutarate:oxygen oxidoreductase (3-hydroxylating). Other names in common use include trimethyllysine alpha-ketoglutarate dioxygenase, TML-alpha-ketoglutarate dioxygenase, TML hydroxylase, 6-N,6-N,6-N-trimethyl-L-lysine,2-oxoglutarate:oxygen oxidoreductase, and (3-hydroxylating). This enzyme participates in lysine degradation and L-carnitine biosynthesis and requires the presence of iron and ascorbate.

See also

Related Research Articles

In enzymology, a 2'-deoxymugineic-acid 2'-dioxygenase (EC 1.14.11.24) is an enzyme that catalyzes the chemical reaction

In enzymology, a desacetoxyvindoline 4-hydroxylase (EC 1.14.11.20) is an enzyme that catalyzes the chemical reaction

In enzymology, a deoxyhypusine monooxygenase (EC 1.14.99.29) is an enzyme that catalyzes the chemical reaction

In enzymology, a flavanone 3-dioxygenase (EC 1.14.11.9) is an enzyme that catalyzes the chemical reaction

In enzymology, a flavonol synthase is an enzyme that catalyzes the following chemical reaction :

<span class="mw-page-title-main">Gamma-butyrobetaine dioxygenase</span> Protein-coding gene in the species Homo sapiens

Gamma-butyrobetaine dioxygenase is an enzyme that in humans is encoded by the BBOX1 gene. Gamma-butyrobetaine dioxygenase catalyses the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthesis pathway. Carnitine is essential for the transport of activated fatty acids across the mitochondrial membrane during mitochondrial beta oxidation. In humans, gamma-butyrobetaine dioxygenase can be found in the kidney (high), liver (moderate), and brain. BBOX1 has recently been identified as a potential cancer gene based on a large-scale microarray data analysis.

In enzymology, a gibberellin 2beta-dioxygenase (EC 1.14.11.13) is an enzyme that catalyzes the chemical reaction

In enzymology, a gibberellin 3beta-dioxygenase (EC 1.14.11.15) is an enzyme that catalyzes the chemical reaction

In enzymology, a hyoscyamine (6S)-dioxygenase (EC 1.14.11.11) is an enzyme that catalyzes the chemical reaction

<span class="mw-page-title-main">L-lysine 6-monooxygenase (NADPH)</span> Class of enzymes

In enzymology, a L-lysine 6-monooxygenase (NADPH) (EC 1.14.13.59) is an enzyme that catalyzes the chemical reaction

In enzymology, a peptide-aspartate beta-dioxygenase (EC 1.14.11.16), a member of the alpha-ketoglutarate-dependent hydroxylases superfamily, is an enzyme that catalyzes the chemical reaction

In enzymology, a procollagen-proline 3-dioxygenase (EC 1.14.11.7) is an enzyme that catalyzes the chemical reaction

In enzymology, a proline 3-hydroxylase (EC 1.14.11.28) is an enzyme that catalyzes the chemical reaction

In enzymology, a pyrimidine-deoxynucleoside 1'-dioxygenase (EC 1.14.11.10) is an enzyme that catalyzes the chemical reaction

In enzymology, a pyrimidine-deoxynucleoside 2'-dioxygenase (EC 1.14.11.3) is an enzyme that catalyzes the chemical reaction

In enzymology, a taurine dioxygenase (EC 1.14.11.17) is an enzyme that catalyzes the chemical reaction.

In enzymology, a thymine dioxygenase (EC 1.14.11.6) is an enzyme that catalyzes the chemical reaction

<span class="mw-page-title-main">TMLHE</span> Protein-coding gene in the species Homo sapiens

Trimethyllysine dioxygenase, mitochondrial is an enzyme that in humans is encoded by the TMLHE gene in chromosome X. Mutations in the TMLHE gene resulting in carnitine biosynthesis disruption have been associated with autism symptoms.

Carnitine biosynthesis is a method for the endogenous production of L-carnitine, a molecule that is essential for energy metabolism. In humans and many other animals, L-carnitine is obtained from both diet and by biosynthesis. The carnitine biosynthesis pathway is highly conserved among many eukaryotes and some prokaryotes.

Alpha-ketoglutarate-dependent hydroxylases are a major class of non-heme iron proteins that catalyse a wide range of reactions. These reactions include hydroxylation reactions, demethylations, ring expansions, ring closures, and desaturations. Functionally, the αKG-dependent hydroxylases are comparable to cytochrome P450 enzymes. Both use O2 and reducing equivalents as cosubstrates and both generate water.

References