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	~~'''~~Methylmalonic acid''' (MMA) is a [[dicarboxylic acid]] that is a vital intermediate in the [[metabolism]] of [[fat]] and [[protein]]. It is a substance that is produced when proteins, specifically those containing the [[amino acid]] [[methionine]], are broken down in the body.		{{DISPLAYTITLE:Methylmalonic acid}}

	==~~Chemistry~~==		== Methylmalonic Acid ==
	Methylmalonic acid is a ~~chiral compound with two enantiomers, (R)-~~ and ~~(S)-methylmalonic acid~~. It is a ~~colorless~~, ~~crystalline solid that~~ is ~~soluble in water. The~~ [[~~chemical formula~~]] ~~for methylmalonic acid is C4H6O4~~.		[[File:Propionate_pathway.svg\|thumb\|right\|300px\|Diagram of the propionate pathway, showing the role of methylmalonic acid.]]
			'''Methylmalonic acid''' (MMA) is a dicarboxylic acid that is a key intermediate in the metabolism of certain amino acids and lipids. It is a structural isomer of succinic acid and plays a crucial role in the [[propionate pathway]], which is important for the metabolism of odd-chain fatty acids and certain amino acids such as [[valine]], [[isoleucine]], [[methionine]], and [[threonine]].

	==~~Biosynthesis~~==		== Biochemical Role ==
	Methylmalonic acid is ~~produced~~ in ~~the body during the metabolism of certain amino acids and odd-chain fatty acids. The process begins with~~ the conversion of ~~these substances into [[propionyl-CoA]], which is then converted into methylmalonyl-CoA by the enzyme [[~~propionyl-CoA ~~carboxylase]]. Methylmalonyl-CoA is then converted into~~ succinyl-CoA, a ~~key intermediate~~ in the [[citric acid cycle]]~~, by~~ the enzyme [[methylmalonyl-CoA mutase]].		Methylmalonic acid is involved in the conversion of propionyl-CoA to succinyl-CoA, a critical step in the [[citric acid cycle]]. This conversion requires the enzyme [[methylmalonyl-CoA mutase]], which is dependent on [[vitamin B12]] (cobalamin) as a cofactor. In the absence of adequate vitamin B12, methylmalonic acid accumulates in the blood, leading to a condition known as [[methylmalonic acidemia]].

	==Clinical ~~significance~~==		== Clinical Significance ==
	Elevated levels of methylmalonic acid in the blood~~, a condition known as [[methylmalonic acidemia]],~~ can be ~~a sign~~ of ~~several different health problems, including [[~~vitamin B12 deficiency]], [[~~kidney disease~~]], and ~~certain [[genetic~~ disorders]]. Methylmalonic acidemia is a ~~serious~~ condition ~~that~~ can lead to developmental delays~~, failure to thrive~~, and other health ~~problems if not treated promptly~~.		Elevated levels of methylmalonic acid in the blood or urine can be indicative of vitamin B12 deficiency, which can lead to [[megaloblastic anemia]] and neurological disorders. Methylmalonic acidemia is a metabolic disorder that can result from genetic defects in the enzymes involved in the conversion of methylmalonyl-CoA to succinyl-CoA. This condition can lead to severe metabolic acidosis, developmental delays, and other serious health issues.

	==~~See also~~==		== Diagnostic Use ==
	* [[Propionic acidemia]]		The measurement of methylmalonic acid levels is used as a diagnostic tool to assess vitamin B12 status. Elevated levels of MMA are often used to confirm a diagnosis of vitamin B12 deficiency, especially in cases where serum vitamin B12 levels are borderline or normal.
	* [[Methylmalonic aciduria]]
	* [[Methylmalonyl-CoA mutase deficiency]]

	==~~References~~==		== Related Pathways ==
	~~<references />~~		Methylmalonic acid is part of the broader [[propionate metabolism]] pathway. This pathway is essential for the breakdown of odd-chain fatty acids and certain amino acids, ultimately feeding into the citric acid cycle for energy production.

			== Related Pages ==
			* [[Vitamin B12]]
			* [[Methylmalonyl-CoA mutase]]
			* [[Propionate metabolism]]
			* [[Methylmalonic acidemia]]
			* [[Citric acid cycle]]

	~~[[Category:Dicarboxylic acids]]~~
	~~[[Category:Metabolism]]~~
	[[Category:Biochemistry]]		[[Category:Biochemistry]]
	~~{{Chemistry-stub}}~~		[[Category:Metabolic pathways]]
	~~{{Medicine-stub}}~~		[[Category:Organic acids]]

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'''Methylmalonic acid''' (MMA) is a [[dicarboxylic acid]] that is a vital intermediate in the [[metabolism]] of [[fat]] and [[protein]]. It is a substance that is produced when proteins, specifically those containing the [[amino acid]] [[methionine]], are broken down in the body.

==Chemistry==
Methylmalonic acid is a chiral compound with two enantiomers, (R)- and (S)-methylmalonic acid. It is a colorless, crystalline solid that is soluble in water. The [[chemical formula]] for methylmalonic acid is C4H6O4.

==Biosynthesis==
Methylmalonic acid is produced in the body during the metabolism of certain amino acids and odd-chain fatty acids. The process begins with the conversion of these substances into [[propionyl-CoA]], which is then converted into methylmalonyl-CoA by the enzyme [[propionyl-CoA carboxylase]]. Methylmalonyl-CoA is then converted into succinyl-CoA, a key intermediate in the [[citric acid cycle]], by the enzyme [[methylmalonyl-CoA mutase]].

==Clinical significance==
Elevated levels of methylmalonic acid in the blood, a condition known as [[methylmalonic acidemia]], can be a sign of several different health problems, including [[vitamin B12 deficiency]], [[kidney disease]], and certain [[genetic disorders]]. Methylmalonic acidemia is a serious condition that can lead to developmental delays, failure to thrive, and other health problems if not treated promptly.

==See also==
* [[Propionic acidemia]]
* [[Methylmalonic aciduria]]
* [[Methylmalonyl-CoA mutase deficiency]]

==References==
<references />

[[Category:Dicarboxylic acids]]
[[Category:Metabolism]]
[[Category:Biochemistry]]
{{Chemistry-stub}}
{{Medicine-stub}}

Methylmalonic acid - Revision history

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