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Carnitine decarboxylase information


carnitine decarboxylase
Identifiers
EC no.4.1.1.42
CAS no.37237-38-0
Databases
IntEnzIntEnz view
BRENDABRENDA entry
ExPASyNiceZyme view
KEGGKEGG entry
MetaCycmetabolic pathway
PRIAMprofile
PDB structuresRCSB PDB PDBe PDBsum
Gene OntologyAmiGO / QuickGO
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PMCarticles
PubMedarticles
NCBIproteins

The enzyme carnitine decarboxylase (EC 4.1.1.42) catalyzes the chemical reaction

carnitine 2-methylcholine + CO2

This enzyme belongs to the family of lyases, specifically the carboxy-lyases, which cleave carbon-carbon bonds. The systematic name of this enzyme class is carnitine carboxy-lyase (2-methylcholine-forming). This enzyme is also called carnitine carboxy-lyase.[citation needed] It employs one cofactor, ATP.

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Carnitine decarboxylase

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The enzyme carnitine decarboxylase (EC 4.1.1.42) catalyzes the chemical reaction carnitine ⇌ {\displaystyle \rightleftharpoons } 2-methylcholine + CO2...

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Inborn error of lipid metabolism

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Malonyl-CoA decarboxylase deficiency - Malonyl-CoA decarboxylase Primary carnitine deficiency - SLC22A5 (carnitine transporter) Carnitine-acylcarnitine...

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Lysine

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polypeptides, uptake of essential mineral nutrients, and in the production of carnitine, which is key in fatty acid metabolism. Lysine is also often involved...

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Malonic aciduria

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Malonic aciduria or malonyl-CoA decarboxylase deficiency (MCD) is an autosomal-recessive metabolic disorder caused by a genetic mutation that disrupts...

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Crotonase family

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menaquinone (vitamin K2). Carnitine racemase (gene caiD), which catalyses the reversible conversion of crotonobetaine to L-carnitine in Escherichia coli. Methylmalonyl...

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Ketogenesis

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oxidation, switches off acetyl-CoA carboxylase, turns on malonyl-CoA decarboxylase, and consequently induces ketogenesis. Ethanol is a potent AMPK inhibitor...

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Fatty acid metabolism

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membranes. Acyl-carnitine is shuttled inside by a carnitine-acylcarnitine translocase, as a carnitine is shuttled outside. Acyl-carnitine is converted back...

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List of causes of hypoglycemia

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Carbohydrate-deficient glycoprotein syndrome type 1b Carnitine palmitoyltransferase I deficiency Carnitine-acylcarnitine translocase deficiency Chloramphenicol...

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Valproate

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Igisu H (July 1993). "Comparison of the effects of L-carnitine, D-carnitine and acetyl-L-carnitine on the neurotoxicity of ammonia". Biochemical Pharmacology...

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Betaine transporter

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The Betaine/Carnitine/Choline Transporter (BCCT) family proteins are found in Gram-negative and Gram-positive bacteria and archaea. The BCCT family members...

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Randle cycle

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converted to malonyl-CoA by acetyl-CoA carboxylase. Malonyl-CoA inhibits the carnitine palmitoyltransferase (CPT) that controls the entry and oxidation of LCFA...

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Methylmalonic acidemias

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isomer of carnitine as the improper breakdown of the affected substances results in sufferers developing a carnitine deficiency. The carnitine also assists...

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Ataxia

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5-hydroxytryptophan (5-HTP), idebenone, amantadine, physostigmine, L-carnitine or derivatives, trimethoprim/sulfamethoxazole, vigabatrin, phosphatidylcholine...

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Cat food

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An important component of many weight loss/weight control diets is L-carnitine. This is a vitamin-like substance that is found in animal protein. It...

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Coenzyme A

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decarboxylated to 4′-phosphopantetheine by phosphopantothenoylcysteine decarboxylase (PPC-DC; CoaC) 4′-phosphopantetheine is adenylated (or more properly...

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Dog food

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vitamin E and L-carnitine. Vitamin E acts as an antioxidant, which can prevent oxidative damage that occurs during aging. L-carnitine is used to improve...

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Amino acid

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acids are known. Those either are not found in proteins (for example carnitine, GABA, levothyroxine) or are not produced directly and in isolation by...

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Chromosome 1

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UMP-CMP kinase COL16A1 (1p35) COL11A1: collagen, type XI, alpha 1 CPT2: carnitine palmitoyltransferase II CRYZ: Crystallin zeta CSDE1: Cold shock domain...

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Choline

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decarboxylation of serine into ethanolamine, which is catalyzed by a serine decarboxylase. The synthesis of choline from ethanolamine may take place in three...

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Combined malonic and methylmalonic aciduria

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clinical or biochemical effects. One study also mentions treatment with L-carnitine in patients with CMAMMA, but only retrospectively and without mentioning...

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Amphetamine

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transporter that is present in astrocytes, and SLC22A5 is a high-affinity carnitine transporter. Amphetamine is known to strongly induce cocaine- and amphetamine-regulated...

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List of enzymes

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Category:EC 4.1.1 Ornithine decarboxylase (EC 4.1.1.17) Uridine monophosphate synthetase (EC 4.1.1.23) Aromatic-L-amino-acid decarboxylase (EC 4.1.1.28) RubisCO...

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Transporter Classification Database

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Uptake (Dcu) Family 2.A.14 Lactate Permease Family 2.A.15 The Betaine/Carnitine/Choline Transporter (BCCT) Family 2.A.16 Tellurite-resistance/Dicarboxylate...

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List of OMIM disorder codes

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complex variant; 608837; MYH8 Carney complex, type 1; 160980; PRKAR1A Carnitine deficiency, systemic primary; 212140; SLC22A5 Carotid intimal medial thickness...

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