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gamma-Butyrobetaine DioxygenaseCarnitineBetaineMixed Function OxygenasesAscorbic Acid DeficiencyLysineWhole-Body CountingDioxygenasesCarnitine O-PalmitoyltransferaseLiver4-Hydroxyphenylpyruvate DioxygenaseOxygenasesCarnitine O-AcetyltransferaseCatechol 2,3-DioxygenaseProtocatechuate-3,4-DioxygenaseCysteine DioxygenaseCarnitine AcyltransferasesCatechol 1,2-DioxygenaseTryptophan OxygenaseHomogentisate 1,2-Dioxygenase3-Hydroxyanthranilate 3,4-DioxygenaseAcetylcarnitineKynurenine

HydroxylaseSuccinateHydroxylationCofactorInhibitorsCrystalStepStudiesTransportInborn error of mOxygenasesTrimethyllysineEnzyme that catalyzesPathwayMitochondrialDietary intakeFatty acidsMetabolism

Hydroxylase6

• 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)
• Crystal structure of human gamma-butyrobetaine hydroxylase. (rhea-db.org)
• Gamma-butyrobetaine hydroxylase (GBBH) is a 2-ketoglutarate-dependent dioxygenase that catalyzes the biosynthesis of l-carnitine by hydroxylation of gamma-butyrobetaine (GBB). (rhea-db.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)

Succinate1

• 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)

Hydroxylation1

• Gamma-butyrobetaine dioxygenase is unique among other human 2OG oxygenases that it catalyses both hydroxylation (e.g. (wikipedia.org)

Cofactor1

• Iron is a cofactor for gamma-butyrobetaine dioxygenase. (wikipedia.org)

Inhibitors2

• Similar to other 2OG oxygenases, gamma-butyrobetaine dioxygenase can be inhibited by 2OG mimics and aromatic inhibitors such as pyridine 2,4-dicarboxylate. (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)

Crystal1

• A crystal structure of mldronate in complex with gamma-butyrobetaine dioxygenase was published, and it suggests mildronate bind to gamma-butyrobetaine dioxygenase in exactly the same way as gamma-butyrobetaine (PDB id: 3MS5). (wikipedia.org)

Step1

• Gamma-butyrobetaine dioxygenase catalyses the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthesis pathway. (wikipedia.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)

Transport2

• Carnitine is essential for the transport of activated fatty acids across the mitochondrial membrane during mitochondrial beta oxidation. (wikipedia.org)
• l-carnitine is required for the transport of long-chain fatty acids into mitochondria for generating metabolic energy. (rhea-db.org)

Inborn error of m1

• This deletion leads to a novel inborn error of metabolism in carnitine biosynthesis. (medscape.com)

Oxygenases3

• Similar to many other 2OG oxygenases, the activity of gamma-butyrobetaine dioxygenase can be stimulated by reducing agents such as ascorbate and glutathione. (wikipedia.org)
• Gamma-butyrobetaine dioxygenase is unique among other human 2OG oxygenases that it catalyses both hydroxylation (e.g. (wikipedia.org)
• Similar to other 2OG oxygenases, gamma-butyrobetaine dioxygenase can be inhibited by 2OG mimics and aromatic inhibitors such as pyridine 2,4-dicarboxylate. (wikipedia.org)

Trimethyllysine1

• Along the way, trimethyllysine (TML) is converted to 3-hydroxy-TML via the mitochondrial enzyme, trimethyllysine dioxygenase (TMLD). (medscape.com)

Enzyme that catalyzes1

• 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)

Pathway2

• Gamma-butyrobetaine dioxygenase catalyses the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthesis pathway. (wikipedia.org)
• use DICARBOXYLIC ACIDS 1970-1979 MH - 3-Phosphoshikimate 1-Carboxyvinyltransferase UI - D051229 MN - D8.811.913.225.735 MS - An enzyme of the shikimate pathway of AROMATIC AMINO ACID biosynthesis, it generates 5-enolpyruvylshikimate 3-phosphate and ORTHOPHOSPHATE from PHOSPHOENOLPYRUVATE and SHIKIMATE-3-PHOSPHATE. (nih.gov)

Mitochondrial1

• Carnitine is essential for the transport of activated fatty acids across the mitochondrial membrane during mitochondrial beta oxidation. (wikipedia.org)

Dietary intake2

• However, she pointed out, "this is likely due to dietary intake of carnitine, and we don't know what carnitine levels are in the brains of these 2 boys [or]…what their carnitine levels were when they were young infants. (medscape.com)
• Renal carnitine reabsorption along with dietary intake and endogenous production maintain carnitine homeostasis. (nih.gov)

Fatty acids1

• As Dr. Celestino-Soper observed, carnitine is used in the transport of fatty acids into the mitochondria, which are broken down for energy production. (medscape.com)

Metabolism1

• Carnitine plays essential roles in intermediary metabolism. (nih.gov)