Acyl-CoA synthetase

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Template:Short description Acyl-CoA synthetases, also known as acyl-CoA ligases, are enzymes that “activate” fatty acids by thioesterification to CoA.[1] It represents the initial step of fatty acid metabolism so that fatty acids can participate in catabolic and anabolic pathways.[1] Among these are, for example, the synthesis of triacylglycerol, phospholipids, plasmalogens, sphingolipids, the degradation of fatty acids for energy production, the conversion to alcohols or aldehydes, the elongation of fatty acids, the insertion and removal of double bonds or the covalent binding to proteins.[1][2]

The members of this family mainly activate fatty acids, but there are also members that activate other substrates instead, such as AACS, which activates acetoacetic acid, or ACSF3, which activates methylmalonic acid and malonic acid.[3][4]

Reaction

Acyl-CoA synthetases catalyze fatty acid activation, which consists of 2 steps:[1]

First, an ATP-dependent adenylation and release of pyrophophates takes place.[1]

Fatty acid+ATP → Acyl−AMP+PPi

In the 2nd step, CoA-SH displaces the adenylate group, which is released as AMP, and forms a thioester bond with the substrate.[1]

Acyl−AMP+CoA−SH → Acyl−S−CoA+AMP

In summary:

Fatty acid+CoA+ATP → Acyl−CoA+AMP+PPi

Members

Symbol Name Localization EC number Preferred substrate Product Gene defect Prevalence
colspan="8" Template:Rh | Short-chain acyl-CoA synthetase family[1]
ACSS1[5] Acyl-CoA synthetase short-chain family member 1 Mitochondrion[5]

EC 6.2.1.1[5] EC 6.2.1.17[5]

Acetate[5] Acetyl-CoA[5]
ACSS2[6] Acyl-CoA synthetase short-chain family member 2 Cytosol[6]

EC 6.2.1.1[6] EC 6.2.1.17[6]

Acetate[6] Acetyl-CoA[6]
ACSS3[7] Acyl-CoA synthetase short-chain family member 3 Cytosol, mitochondrial matrix[7]

EC 6.2.1.1[7] EC 6.2.1.17[7]

Acetate Acetyl-CoA
colspan="8" Template:Rh | Medium-chain acyl-CoA synthetase family[1] (C6-C10)
ACSM1[8] Acyl-CoA synthetase medium-chain family member 1 Mitochondrial matrix[8]

EC 6.2.1.2[8] EC 6.2.1.25[8]

ACSM2A[9] Acyl-CoA synthetase medium-chain family member 2A Mitochondrion[9]

EC 6.2.1.2[9] EC 6.2.1.25[9]

Medium chain fatty acids
ACSM2B[10] Acyl-CoA synthetase medium-chain family member 2B Mitochondrion[10]

EC 6.2.1.2[10] EC 6.2.1.25[10]

ACSM3[11] Acyl-CoA synthetase medium-chain family member 3 Mitochondrion[11]

EC 6.2.1.17[11] EC 6.2.1.2[11]

ACSM4[12] Acyl-CoA synthetase medium-chain family member 4 Mitochondrial matrix (predicted)[12]

EC 6.2.1.2[12]

Decanoate-CoA (predicted)[12]
ACSM5[13] Acyl-CoA synthetase medium-chain family member 5 Mitochondrial matrix (predicted)[13]

EC 6.2.1.2[13]

ACSM6[14] Acyl-CoA synthetase medium-chain family member 6 Mitochondrial Matrix (predicted)[14]

EC 6.2.1.2[14]

colspan="8" Template:Rh | Long-chain acyl-CoA synthetase family[1]
ACSL1[15] Acyl-CoA synthetase long-chain family member 1

EC 6.2.1.15[15] EC 6.2.1.24[15] EC 6.2.1.3[15]

Free long-chain fatty acids[15] Fatty acyl-CoA esters[15]
ACSL3[16] Acyl-CoA synthetase long-chain family member 3

EC 6.2.1.15[16] EC 6.2.1.2[16] EC 6.2.1.3[16]

Myristate,[16] arachidonate,[16] eicosapentaenoate[16]
ACSL4[17] Acyl-CoA synthetase long-chain family member 4 Peroxisome[2]

EC 6.2.1.15[17] EC 6.2.1.3[17]

Arachidonate[17] Intellectual developmental disorder, X-linked 63[18]
ACSL5[19] Acyl-CoA synthetase long-chain family member 5

EC 6.2.1.15[19] EC 6.2.1.3[19]

ACSL6[20] Acyl-CoA synthetase long-chain family member 6

EC 6.2.1.15[20] EC 6.2.1.3[20]

colspan="8" Template:Rh | Very long-chain acyl-CoA synthetase family[1] (>C22)
SLC27A1[21] Solute carrier family 27 (fatty acid transporter), member 1 Plasma membrane,[21] Endoplasmic reticulum

EC 6.2.1.15[21] EC 6.2.1.3[21]

SLC27A2[22] Solute carrier family 27 (fatty acid transporter), member 2 Endoplasmic reticulum,[22] peroxisome[22][2]

EC 6.2.1.15[22] EC 6.2.1.24[22] EC 6.2.1.3[22] EC 6.2.1.7[22]

Long-chain, branched-chain and very long-chain fatty acids[22] Fatty acyl-CoA esters[22]
SLC27A3[23] Solute carrier family 27 (fatty acid transporter), member 3

EC 6.2.1.15[23] EC 6.2.1.3[23]

SLC27A4[24] Solute carrier family 27 (fatty acid transporter), member 4 Peroxisome[2]

EC 6.2.1.15[24] EC 6.2.1.3[24]

Ichthyosis prematurity syndrome[24]
SLC27A5[25] Solute carrier family 27 (fatty acid transporter), member 5 Endoplasmic reticulum[25]

EC 6.2.1.3[25] EC 6.2.1.7[25]

Very long-chain fatty-acids containing 24- and 26-carbons[25]
SLC27A6[26] Solute carrier family 27 (fatty acid transporter), member 6

EC 6.2.1.15[26] EC 6.2.1.3[26]

colspan="8" Template:Rh | Bubblegum acyl-CoA synthetase family[1]
ACSBG1[27] Acyl-CoA synthetase bubblegum family member 1

EC 6.2.1.3[27]

ACSBG2[28] Acyl-CoA synthetase bubblegum family member 2 Cytosol,[28] Mitochondrion[28]

EC 6.2.1.15[28] EC 6.2.1.3[28]

colspan="8" Template:Rh | Other acyl-CoA synthetases [1]
AACS[3] Acetoacetyl-CoA synthetase Cytosol (predicted)[3]

EC 6.2.1.16[3]

Acetoacetate[3] Acetoacetyl-CoA (predicted)[3]
ACSF2[29] Acyl-CoA synthetase family member 2 Mitochondrion[29]

EC 6.2.1.2[29]

ACSF3[4] Acyl-CoA synthetase family member 3 Mitochondrion[4]

EC 6.2.1.76[4]

Malonate,[4] methylmalonate[4] Malonyl-CoA, methylmalonyl-CoA Combined malonic and methylmalonic aciduria (CMAMMA)[4] 1: 30,000[30]
AASDH[31] Aminoadipate-semialdehyde dehydrogenase

EC 6.2.1.-[31]

References

Template:Reflist

Template:Ligases CO CS and CN Template:Enzymes Template:Portal bar

Template:Improve categories Template:Ligase-stub

  1. ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 Template:Cite journal
  2. ↑ 2.0 2.1 2.2 2.3 Template:Cite journal
  3. ↑ 3.0 3.1 3.2 3.3 3.4 3.5 Template:Cite web
  4. ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 Template:Cite web
  5. ↑ 5.0 5.1 5.2 5.3 5.4 5.5 Template:Cite web
  6. ↑ 6.0 6.1 6.2 6.3 6.4 6.5 Template:Cite web
  7. ↑ 7.0 7.1 7.2 7.3 Template:Cite web
  8. ↑ 8.0 8.1 8.2 8.3 Template:Cite web
  9. ↑ 9.0 9.1 9.2 9.3 Template:Cite web
  10. ↑ 10.0 10.1 10.2 10.3 Template:Cite web
  11. ↑ 11.0 11.1 11.2 11.3 Template:Cite web
  12. ↑ 12.0 12.1 12.2 12.3 Template:Cite web
  13. ↑ 13.0 13.1 13.2 Template:Cite web
  14. ↑ 14.0 14.1 14.2 Template:Cite web
  15. ↑ 15.0 15.1 15.2 15.3 15.4 15.5 Template:Cite web
  16. ↑ 16.0 16.1 16.2 16.3 16.4 16.5 16.6 Template:Cite web
  17. ↑ 17.0 17.1 17.2 17.3 Template:Cite web
  18. ↑ Template:Cite web
  19. ↑ 19.0 19.1 19.2 Template:Cite web
  20. ↑ 20.0 20.1 20.2 Template:Cite web
  21. ↑ 21.0 21.1 21.2 21.3 Template:Cite web
  22. ↑ 22.0 22.1 22.2 22.3 22.4 22.5 22.6 22.7 22.8 Template:Cite web
  23. ↑ 23.0 23.1 23.2 Template:Cite web
  24. ↑ 24.0 24.1 24.2 24.3 Template:Cite web
  25. ↑ 25.0 25.1 25.2 25.3 25.4 Template:Cite web
  26. ↑ 26.0 26.1 26.2 Template:Cite web
  27. ↑ 27.0 27.1 Template:Cite web
  28. ↑ 28.0 28.1 28.2 28.3 28.4 Template:Cite web
  29. ↑ 29.0 29.1 29.2 Template:Cite web
  30. ↑ Template:Cite journal
  31. ↑ 31.0 31.1 Template:Cite web
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