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Methylmalonyl CoA epimerase

Index Methylmalonyl CoA epimerase

Methylmalonyl CoA epimerase (methylmalonyl-CoA racemase, methylmalonyl coenzyme A racemase, DL-methylmalonyl-CoA racemase, 2-methyl-3-oxopropanoyl-CoA 2-epimerase) is an enzyme involved in fatty acid catabolism that is encoded in human by the "MCEE" gene located on chromosome 2. [1]

34 relations: Acetyl-CoA, Beta oxidation, Branched-chain amino acid, Carbanion, Catalysis, Chemical reaction, Chromosome 2, Cofactor (biochemistry), Coma, Dominance (genetics), Dysphagia, Enzyme, Enzyme catalysis, Exon, Fatty acid, Gene, Hyperammonemia, Inborn errors of metabolism, Isoleucine, Leucine, Metabolic acidosis, Methylmalonyl-CoA, Methylmalonyl-CoA mutase, Mitochondrial matrix, Mutation, Odd-chain fatty acid, Propionyl-CoA carboxylase, Protein, Reactive intermediate, Saturation (chemistry), Succinyl-CoA, Valine, Vitamin B12, X-ray crystallography.

Acetyl-CoA

Acetyl-CoA (acetyl coenzyme A) is a molecule that participates in many biochemical reactions in protein, carbohydrate and lipid metabolism.

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Beta oxidation

In biochemistry and metabolism, beta-oxidation is the catabolic process by which fatty acid molecules are broken down in the cytosol in prokaryotes and in the mitochondria in eukaryotes to generate acetyl-CoA, which enters the citric acid cycle, and NADH and FADH2, which are co-enzymes used in the electron transport chain.

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Branched-chain amino acid

A branched-chain amino acid (BCAA) is an amino acid having aliphatic side-chains with a branch (a central carbon atom bound to three or more carbon atoms).

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Carbanion

A carbanion is an anion in which carbon is threevalent (forms three bonds) and bears a formal negative charge in at least one significant mesomeric contributor (resonance form).

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Catalysis

Catalysis is the increase in the rate of a chemical reaction due to the participation of an additional substance called a catalysthttp://goldbook.iupac.org/C00876.html, which is not consumed in the catalyzed reaction and can continue to act repeatedly.

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Chemical reaction

A chemical reaction is a process that leads to the transformation of one set of chemical substances to another.

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Chromosome 2

Chromosome 2 is one of the 23 pairs of chromosomes in humans.

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Cofactor (biochemistry)

A cofactor is a non-protein chemical compound or metallic ion that is required for an enzyme's activity.

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Coma

Coma is a state of unconsciousness in which a person cannot be awaken; fails to respond normally to painful stimuli, light, or sound; lacks a normal wake-sleep cycle; and does not initiate voluntary actions.

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Dominance (genetics)

Dominance in genetics is a relationship between alleles of one gene, in which the effect on phenotype of one allele masks the contribution of a second allele at the same locus.

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Dysphagia

Dysphagia is the medical term for the symptom of difficulty in swallowing.

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Enzyme

Enzymes are macromolecular biological catalysts.

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Enzyme catalysis

Enzyme catalysis is the increase in the rate of a chemical reaction by the active site of a protein.

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Exon

An exon is any part of a gene that will encode a part of the final mature RNA produced by that gene after introns have been removed by RNA splicing.

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Fatty acid

In chemistry, particularly in biochemistry, a fatty acid is a carboxylic acid with a long aliphatic chain, which is either saturated or unsaturated.

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Gene

In biology, a gene is a sequence of DNA or RNA that codes for a molecule that has a function.

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Hyperammonemia

Hyperammonemia (or hyperammonaemia) is a metabolic disturbance characterised by an excess of ammonia in the blood.

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Inborn errors of metabolism

Inborn errors of metabolism form a large class of genetic diseases involving congenital disorders of metabolism.

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Isoleucine

Isoleucine (symbol Ile or I) is an α-amino acid that is used in the biosynthesis of proteins.

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Leucine

Leucine (symbol Leu or L) is an essential amino acid that is used in the biosynthesis of proteins.

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Metabolic acidosis

Metabolic acidosis is a condition that occurs when the body produces excessive quantities of acid or when the kidneys are not removing enough acid from the body.

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Methylmalonyl-CoA

Methylmalonyl-CoA is the thioester consisting of coenzyme A linked to methylmalonic acid.

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Methylmalonyl-CoA mutase

Methylmalonyl-CoA mutase (MCM), mitochondrial, also known as methylmalonyl-CoA isomerase, is a protein that in humans is encoded by the MUT gene.

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Mitochondrial matrix

In the mitochondrion, the matrix is the space within the inner membrane.

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Mutation

In biology, a mutation is the permanent alteration of the nucleotide sequence of the genome of an organism, virus, or extrachromosomal DNA or other genetic elements.

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Odd-chain fatty acid

Odd chain fatty acids are those that contain an odd number of carbon atoms in the structure.

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Propionyl-CoA carboxylase

Propionyl-CoA carboxylase catalyses the carboxylation reaction of propionyl CoA in the mitochondrial matrix.

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Protein

Proteins are large biomolecules, or macromolecules, consisting of one or more long chains of amino acid residues.

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Reactive intermediate

In chemistry, a reactive intermediate or an intermediate is a short-lived, high-energy, highly reactive molecule.

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Saturation (chemistry)

In chemistry, saturation (from the Latin word saturare, meaning 'to fill') has diverse meanings, all based on the idea of reaching a maximum capacity.

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Succinyl-CoA

Succinyl-Coenzyme A, abbreviated as Succinyl-CoA or SucCoA, is a combination of succinic acid and coenzyme A.

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Valine

Valine (symbol Val or V) is an α-amino acid that is used in the biosynthesis of proteins.

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Vitamin B12

Vitamin B12, also called cobalamin, is a water-soluble vitamin that is involved in the metabolism of every cell of the human body: it is a cofactor in DNA synthesis, and in both fatty acid and amino acid metabolism.

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X-ray crystallography

X-ray crystallography is a technique used for determining the atomic and molecular structure of a crystal, in which the crystalline atoms cause a beam of incident X-rays to diffract into many specific directions.

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Redirects here:

EC 5.1.99.1, Methylmalonyl-CoA 2-epimerase, Methylmalonyl-CoA epimerase, Methylmalonyl-CoA racemase.

References

[1] https://en.wikipedia.org/wiki/Methylmalonyl_CoA_epimerase

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