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carnitine biosynthesis

see Figure Biochemistry: - carnitine biosynthesis 1 - enzyme: methyltransferase - substrate: protein + 3 S-adenosylmethionine - product: protein with lys[CH3]3) + 3 S-adenosylhomocysteine - carnitine biosynthesis 2 - enzyme: protease - substrate: protein with lys[CH3]3 + H2O - product: peptide proteolyticfragment + trimethyllysine - carnitine biosynthesis 3 - enzyme: trimethyllysine hydroxylase - substrate: trimethyllysine + alpha-ketoglutarate + O2 - product: beta-hydroxy trimethyllysine + succinate + CO2 - carnitine biosynthesis 4 - enzyme: beta-hydroxy trimethyllysine aldolase - substrate: beta-hydroxy trimethyllysine - product: gamma-trimethylaminobutyraldehyde + glycine - carnitine biosynthesis 5 - enzyme: aldehyde dehydrogenase - substrate: gamma-trimethylaminobutyraldehyde + NAD + H2O - product: gamma-butyrobetaine + NADH - carnitine biosynthesis 6 - enzyme: gamma-butyrobetaine hydroxylase - substrate: gamma-butyrobetaine + alpha-ketoglutarate + O2 - product: carnitine + succinate + CO2

Related

carnitine (Carnitor)

General

biosynthetic pathway or anabolic pathway

Properties

EVENTS: enzymatic reaction ENZYME: methyltransferase MOTIF: active site SUBSTRATE: protein S-adenosylmethionine (3) PRODUCT: protein MOTIF: lys[CH3]3 S-adenosylhomocysteine (3) TAG: CARNITINE-BIOSYNTHESIS-1 enzymatic reaction ENZYME: protease STATE: active state MOTIF: active site SUBSTRATE: protein MOTIF: lys[CH3]3 H2O PRODUCT: peptide trimethyllysine TAG: CARNITINE-BIOSYNTHESIS-2 enzymatic reaction ENZYME: trimethyllysine hydroxylase COMPARTMENT: mitochondrial matrix CELL: hepatocyte unspecified cell type WITHIN: kidney cardiac muscle skeletal muscle unspecified cell type WITHIN: brain MOTIF: active site metal ion-binding site binding site FOR-BINDING-OF: alpha-ketoglutarate binding site FOR-BINDING-OF: ascorbate SUBSTRATE: trimethyllysine alpha-ketoglutarate O2 PRODUCT: beta-OH trimethyllysine succinate COMPARTMENT: mitochondrial matrix CO2 TAG: CARNITINE-BIOSYNTHESIS-3 enzymatic reaction ENZYME: beta-OH trimethyllysine aldolase MOTIF: active site SUBSTRATE: beta-OH trimethyllysine PRODUCT: gamma-trimethyl amino butyraldehyde glycine TAG: CARNITINE-BIOSYNTHESIS-4 enzymatic reaction ENZYME: aldehyde dehydrogenase MOTIF: active site binding site FOR-BINDING-OF: NAD SUBSTRATE: gamma-trimethyl amino butyraldehyde NAD H2O PRODUCT: gamma-butyrobetaine NADH TAG: CARNITINE-BIOSYNTHESIS-5 enzymatic reaction ENZYME: gamma-butyrobetaine hydroxylase COMPARTMENT: cytoplasm CELL: hepatocyte MOTIF: active site metal ion-binding site binding site FOR-BINDING-OF: alpha-ketoglutarate binding site FOR-BINDING-OF: ascorbate SUBSTRATE: gamma-butyrobetaine alpha-ketoglutarate O2 PRODUCT: carnitine COMPARTMENT: inner mitochondrial membrane succinate COMPARTMENT: mitochondrial matrix CO2 TAG: CARNITINE-BIOSYNTHESIS-6

References

  1. Rebouche CJ. Carnitine function and requirements during the life cycle. FASEB J. 1992 Dec;6(15):3379-86. Review. PMID: 1464372