Gamma-butyrobetaine dioxygenase (also known as BBOX, GBBH or γ-butyrobetaine hydroxylase) is an enzyme that in humans is encoded by the BBOX1 gene. (wikipedia.org)
Structural and mechanistic studies on γ-butyrobetaine hydroxylase. (ox.ac.uk)
The final step in carnitine biosynthesis is catalyzed by γ-butyrobetaine (γBB) hydroxylase (BBOX), an iron/2-oxoglutarate (2OG) dependent oxygenase. (ox.ac.uk)
Carnitinen dannes gennem flere enzymatiske trin, hvor to trin katalyseres af 2-OGDO enzymerne: trimethyllysine hydroxylase ( TMLH ) og gamma-butyrobetaine hydroxylase ( BBOX eller GBBH), hvor Fe2+ er cofactor (3). (blogspot.com)
Natural substrate1
better source needed] Mildronate has a similar structure to the natural substrate gamma-butyrobetaine, with a NH group replacing the CH2 of gamma-butyrobetaine at the C-4 position. (wikipedia.org)
Biosynthesis1
Gamma-butyrobetaine dioxygenase catalyses the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthesis pathway. (wikipedia.org)
It catalyses the following reaction: 4-trimethylammoniobutanoate (γ-butyrobetaine) + 2-oxoglutarate + O2 ⇌ {\displaystyle \rightleftharpoons } 3-hydroxy-4-trimethylammoniobutanoate (L-carnitine) + succinate + CO2 The three substrates of this enzyme are 4-trimethylammoniobutanoate (γ-butyrobetaine), 2-oxoglutarate, and O2, whereas its three products are 3-hydroxy-4-trimethylammoniobutanoate (L-carnitine), succinate, and carbon dioxide. (wikipedia.org)
Carnitine1
Gamma-butyrobetaine dioxygenase is promiscuous in substrate selectivity and it processes a number of modified substrates, including the natural catalytic products L-carnitine and D-carnitine, forming 3-dehydrocarnitine and trimethylaminoacetone. (wikipedia.org)
Metabolism1
2) metabolism of endogenous and a wide variety of exogenous substrates, such as toxins and drugs (BIOTRANSFORMATION). (bvsalud.org)
Studies1
Although initial reports suggested mildronate is a non-competitive and non-hydroxylatable analogue of gamma-butyrobetaine, further studies have identified mildronate is indeed a substrate for gamma-butyrobetaine dioxygenase. (wikipedia.org)
Links1
THP is a substrate giving formaldehyde (supporting structural links with histone demethylases), dimethylamine, malonic acid semi-aldehyde, and an unexpected product with an additional carbon-carbon bond resulting from N-demethylation coupled to oxidative rearrangement, likely via an unusual radical mechanism. (ox.ac.uk)
Enzyme3
It catalyses the following reaction: 4-trimethylammoniobutanoate (γ-butyrobetaine) + 2-oxoglutarate + O2 ⇌ {\displaystyle \rightleftharpoons } 3-hydroxy-4-trimethylammoniobutanoate (L-carnitine) + succinate + CO2 The three substrates of this enzyme are 4-trimethylammoniobutanoate (γ-butyrobetaine), 2-oxoglutarate, and O2, whereas its three products are 3-hydroxy-4-trimethylammoniobutanoate (L-carnitine), succinate, and carbon dioxide. (wikipedia.org)
Other reported gamma-butyrobetaine dioxygenase inhibitors include cyclopropyl-substituted gamma-butyrobetaines and 3-(2,2-dimethylcyclopropyl)propanoic acid, which is a mechanism-based enzyme inhibitor. (wikipedia.org)
use AMINO ACIDS, BRANCHED-CHAIN 1979, & KETO ACIDS & VALERATES 1973-1979 MH - 3-Hydroxyanthranilate 3,4-Dioxygenase UI - D050561 MN - D8.811.682.690.416.328 MS - An enzyme that catalyzes the conversion of 3-hydroxyanthranilate to 2-amino-3-carboxymuconate semialdehyde. (nih.gov)
Oxygen1
These reactions require two substrates as reductants for each of the two oxygen atoms. (bvsalud.org)