Methylcrotonyl-CoA: Difference between revisions

{{dist|MG-CoA}}

| verifiedrevid = 432420444

| ImageFile=Methylcrotonyl coenzyme A.svg

| ImageSize=300px

| IUPACName=3′-''O''-Phosphonoadenosine 5′-[(3''R'')-3-hydroxy-2-methyl-4-{[3-({2-[(3-methylbut-2-enoyl)sulfanyl]ethyl}amino)-3-oxopropyl]amino}-4-oxobutyl dihydrogen diphosphate]

| PIN=''O''¹-<nowiki/>{[(2''R'',3''S'',4''R'',5''R'')-5-(6-Amino-9''H''-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methyl} ''O''³-[(3''R'')-3-hydroxy-2-methyl-4-{[3-({2-[(3-methylbut-2-enoyl)sulfanyl]ethyl}amino)-3-oxopropyl]amino}-4-oxobutyl] dihydrogen diphosphate

| OtherNames=

|Section1={{Chembox Identifiers

| CASNo_Ref =

| CASNo=6712-03-4

| PubChem=439869

| InChI=InChI=1S/C26H42N7O17P3S/c1-14(2)9-17(35)54-8-7-28-16(34)5-6-29-24(38)21(37)26(3,4)11-47-53(44,45)50-52(42,43)46-10-15-20(49-51(39,40)41)19(36)25(48-15)33-13-32-18-22(27)30-12-31-23(18)33/h9,12-13,15,19-21,25,36-37H,5-8,10-11H2,1-4H3,(H,28,34)(H,29,38)(H,42,43)(H,44,45)(H2,27,30,31)(H2,39,40,41)/t15-,19-,20-,21?,25-/m1/s1

| InChIKey=BXIPALATIYNHJN-TVCSPYKZSA-N

| SMILES=CC(=CC(=O)SCCNC(=O)CCNC(=O)C(C(C)(C)COP(=O)(O)OP(=O)(O)OC[C@@H]1[C@H]([C@H]([C@@H](O1)N2C=NC3=C(N=CN=C32)N)O)OP(=O)(O)O)O)C

| MeSHName=Methylcrotonyl-CoA

|Section2={{Chembox Properties

| Formula=C₂₆H₄₂N₇O₁₇P₃S

| MolarMass=849.636 g/mol

| Appearance=

| Density=

| MeltingPt=

| BoilingPt=

| Solubility=

|Section3={{Chembox Hazards

| MainHazards=

| FlashPt=

| AutoignitionPt =

'''3-Methylcrotonyl-CoA''' ('''β-Methylcrotonyl-CoA''' or '''MC-CoA''') is an [[metabolic intermediate|intermediate]] in the metabolism of [[leucine]].<ref name="ISSN position stand 2013" /><ref name="HMB athletic performance-related effects 2011 review">{{cite journal | vauthors = Zanchi NE, Gerlinger-Romero F, Guimarães-Ferreira L, de Siqueira Filho MA, Felitti V, Lira FS, Seelaender M, Lancha AH | display-authors = 6 | title = HMB supplementation: clinical and athletic performance-related effects and mechanisms of action | journal = Amino Acids | volume = 40 | issue = 4 | pages = 1015–1025 | date = April 2011 | pmid = 20607321 | doi = 10.1007/s00726-010-0678-0 | s2cid = 11120110 | url = https://repositorio.unal.edu.co/handle/unal/77957| quote = HMB is a metabolite of the amino acid leucine (Van Koverin and Nissen 1992), an essential amino acid. The first step in HMB metabolism is the reversible transamination of leucine to [α-KIC] that occurs mainly extrahepatically (Block and Buse 1990). Following this enzymatic reaction, [α-KIC] may follow one of two pathways. In the first, HMB is produced from [α-KIC] by the cytosolic enzyme KIC dioxygenase (Sabourin and Bieber 1983). The cytosolic dioxygenase has been characterized extensively and differs from the mitochondrial form in that the dioxygenase enzyme is a cytosolic enzyme, whereas the dehydrogenase enzyme is found exclusively in the mitochondrion (Sabourin and Bieber 1981, 1983). Importantly, this route of HMB formation is direct and completely dependent of liver KIC dioxygenase. Following this pathway, HMB in the cytosol is first converted to cytosolic β-hydroxy-β-methylglutaryl-CoA (HMG-CoA), which can then be directed for cholesterol synthesis (Rudney 1957) (Fig. 1). In fact, numerous biochemical studies have shown that HMB is a precursor of cholesterol (Zabin and Bloch 1951; Nissen et al. 2000).<!--<br /><br /> In the second pathway, after transamination, [α-KIC] in liver generates isovaleryl-CoA through the enzymatic action of branched-chain ketoacid dehydrogenase (BCKD) and after several steps, there is production of HMG-CoA through the enzyme HMG-CoA synthase (Fig. 1). Under normal conditions the majority of KIC is converted into isovaleryl-CoA, in which approximately 5% of leucine is metabolized into HMB (Wilson et al. 2008; Van Koverin and Nissen 1992). However, Nissen and Abumrad (1997) provided evidence that the primary fate of HMB is probably conversion to HMG-CoA in the liver, for cholesterol biosynthesis.--> }}</ref><ref name="Leucine metabolism" />

It is found in mitochondria, where it is formed from [[isovaleryl-coenzyme A]] by [[isovaleryl coenzyme A dehydrogenase]]. It then reacts with CO₂ to yield [[Methylcrotonyl-CoA carboxylase|3-Methylcrotonyl-CoA carboxylase]]. <ref>{{cite journal | vauthors = Grünert SC, Stucki M, Morscher RJ, Suormala T, Bürer C, Burda P, Christensen E, Ficicioglu C, Herwig J, Kölker S, Möslinger D, Pasquini E, Santer R, Schwab KO, Wilcken B, Fowler B, Yue WW, Baumgartner MR | display-authors = 6 | title = 3-methylcrotonyl-CoA carboxylase deficiency: clinical, biochemical, enzymatic and molecular studies in 88 individuals | journal = Orphanet Journal of Rare Diseases | volume = 7 | issue = 1 | pages = 31 | date = May 2012 | pmid = 22642865 | pmc = 3495011 | doi = 10.1186/1750-1172-7-31 | doi-access = free }}</ref>

==Leucine metabolism==

{{Leucine metabolism in humans|align=center}}

== See also ==

* [[Methylcrotonyl-CoA carboxylase]]

== References ==

{{Reflist}}

{{Amino acid metabolism intermediates}}

[[Category:Thioesters of coenzyme A]]

{{biochem-stub}}