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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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Carnitine shuttle

Transfer of long-chain acyl groups across mitochondrial membranes through carnitine-linked intermediates. 11 reactions, from Reactome R-HSA-200425.

  1. OCTN2 / SLC22A5 transports CAR from extracellular space to cytosolCAR + Na+ → CAR + Na+
  2. CPT1A transfers PALM to CARCAR + PALM-CoA → CoA-SH + L-PCAR
  3. CPT2 converts acylcarnitine to acyl-CoACoA-SH + C8-C18 Acyl-CAR → CAR + C8-C18 Acyl-CoA
  4. pAMPK inactivates ACACB, inhibiting malonyl-CoA synthesisBtn-ACACB:2Mn2+ polymer + ATP → Btn-p-S222-ACACB:2Mn2+ polymer + ADP + H+
  5. Exchange of acylcarnitine and carnitine across the inner mitochondrial membraneCAR + Acyl-CAR → CAR + Acyl-CAR
  6. Btn-ACACA:2Mn2+ polymer carboxylates Ac-CoA to form Mal-CoAAc-CoA + ATP + HCO3- → ADP + Pi + Mal-CoA + H+
  7. MID1IP1 binds THRSPMID1IP1 + THRSP → MID1IP1:THRSP
  8. Btn-ACACB:2Mn2+ polymer carboxylates Ac-CoA to form Mal-CoAAc-CoA + ATP + HCO3- → ADP + Pi + Mal-CoA + H+
  9. CAR translocates from the cytosol to the mitochondrial intermembrane spaceCAR → CAR
  10. CPT1B transfers PALM to CARCAR + PALM-CoA → CoA-SH + L-PCAR
  11. SLC25A20 exports excess acyl-CARAcyl-CAR + CAR → Acyl-CAR + CAR