3-sulfino-L-alanine

Three steps

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by SLC25A12, SLC25A13, NAT8L, CPT1A and 2 moremitochondrial matrix, cytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 5 routes

• N-acetyl-L-aspartyl-L-glutamateReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. N-acetyl-L-aspartate + L-glutamate + ATP = N-acetyl-L-aspartyl-L-glutamate + ADP + phosphate + H(+)

  Catalysed by SLC25A12, SLC25A13, NAT8L, RIMKLA and 1 moremitochondrial matrix, cytosol

• N-acetyl-L-aspartyl-L-glutamyl-L-glutamateReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. N-acetyl-L-aspartate + 2 L-glutamate + 2 ATP = N-acetyl-L-aspartyl-L-glutamyl-L-glutamate + 2 ADP + 2 phosphate + 2 H(+)

  Catalysed by SLC25A12, SLC25A13, NAT8L, RIMKLAmitochondrial matrix, cytosol

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by SLC25A12, SLC25A13, NAT8L, CRAT and 1 moremitochondrial matrix, cytosol, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by SLC25A12, SLC25A13, NAT8L, ACAA1 and 3 moremitochondrial matrix, cytosol, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Production of Hexadecanedioylcarnitine

  Catalysed by SLC25A12, SLC25A13, NAT8L, CPT1A and 2 moremitochondrial matrix, cytosolOne of 2 routes

• pristanoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. fatty-acid-CoA ligase

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2mitochondrial matrix, cytosolOne of 2 routes

• undecanoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. medium-chain acyl-CoA ligase (undecylic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, ACSM4 and 5 moremitochondrial matrix, cytosolOne of 3 routes

• (5Z)-tetradecenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (physeteric acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 8 routes

• (3E)-decenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

  Catalysed by SLC25A12, SLC25A13, NAT8L, ACADM and 3 moremitochondrial matrix, cytosol

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

  Catalysed by SLC25A12, SLC25A13, NAT8L, HADHA and 2 moremitochondrial matrix, cytosol

• an N(2)-acyl-L-glutamineReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)
  3. an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)

  Catalysed by SLC25A12, SLC25A13, SLC7A13, GLYATL1B and 1 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane

• (7Z)-hexadecenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 8 routes

• tricosanoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (tricosanoic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 4 routes

• taurochenodeoxycholateReach from here →
  1. 3-sulfino-L-alanine carboxy-lyase
  2. Hypotaurine Oxidase, Extracellular
  3. 3alpha,7alpha-dihydroxy-5beta-cholanoyl-CoA:taurine N-choloyltransferase

  Catalysed by GAD1, CSAD, BAATcytosol, extracellular space, peroxisomeOne of 3 routes

• 2-(1-hydroxyethyl)thiamine diphosphateReach from here →
  1. 3-sulfino-L-alanine:2-oxoglutarate aminotransferase
  2. 3-sulfinylpyruvic acid hydrolysis
  3. pyruvate:thiamin diphosphate acetaldehydetransferase (decarboxylating)

  Catalysed by GOT1, PDHA1, PDHB, PDHA2cytosol, mitochondrial matrix