biocytin

Three steps

• N-acetyl-L-cysteineReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of cysteine and lysine (cytosol to extracellular)
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

  Catalysed by BTD, SLC7A6, NAGSmitochondrial matrix, extracellular space, cytosol

• K(+)Reach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of cysteine and lysine (cytosol to extracellular)
  3. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)

  Catalysed by BTD, SLC7A6, SLC1A1mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• Cys-tRNA(Cys)Reach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of cysteine and lysine (cytosol to extracellular)
  3. L-Cysteine:tRNA(Cys) ligase (AMP-forming)

  Catalysed by BTD, SLC7A6, CARS1mitochondrial matrix, extracellular space, cytosol

• Phe-tRNA(Phe)Reach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of lysine and phenylalanine (cytosol to extracellular)
  3. L-Phenylalanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by BTD, SLC7A6, FARSB, FARSAmitochondrial matrix, extracellular space, cytosol

• Gly-tRNA(Gly)Reach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of glycine and lysine (cytosol to extracellular)
  3. glycine:tRNA(Gly) ligase (AMP-forming)

  Catalysed by BTD, SLC7A6, GARS1mitochondrial matrix, extracellular space, cytosol

• Trp-tRNA(Trp)Reach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of lysine and tryptophan (cytosol to extracellular)
  3. L-Tryptophan -tRNA(Trp) ligase (AMP-forming)

  Catalysed by BTD, SLC7A6, WARS1mitochondrial matrix, extracellular space, cytosol

• Ile-tRNA(Ile)Reach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of isoleucine and lysine (cytosol to extracellular)
  3. L-Isoleucine:tRNA(Ile) ligase (AMP-forming)

  Catalysed by BTD, SLC7A6, IARS1mitochondrial matrix, extracellular space, cytosol

• Gln-tRNA(Gln)Reach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of glutamine and lysine (cytosol to extracellular)
  3. L-Glutamine:tRNA(Gln) ligase (AMP-forming)

  Catalysed by BTD, SLC7A6, QARS1mitochondrial matrix, extracellular space, cytosol

• isocyanateReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of citrulline and lysine (cytosol to extracellular)
  3. L-citrulline + H(+) = isocyanate + L-ornithine

  Catalysed by BTD, SLC7A5, SLC7A6, LACC1mitochondrial matrix, extracellular space, cytosol

• Asn-tRNA(Asn)Reach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of asparagine and lysine (cytosol to extracellular)
  3. L-Asparagine:tRNA(Asn) ligase (AMP-forming)

  Catalysed by BTD, SLC7A6, NARS1mitochondrial matrix, extracellular space, cytosol

• glycocholateReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of glycine and lysine (cytosol to extracellular)
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by BTD, SLC7A6, BAATmitochondrial matrix, extracellular space, cytosol, peroxisomeOne of 3 routes

• hydrogen sulfideReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of cysteine and lysine (cytosol to extracellular)
  3. L-cysteine hydrogen-sulfide-lyase (deaminating

  Catalysed by BTD, SLC7A6, CTHmitochondrial matrix, extracellular space, cytosolOne of 2 routes

• L-aspartateReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of alanine and lysine (cytosol to extracellular)
  3. oxaloacetate + L-alanine = L-aspartate + pyruvate

  Catalysed by BTD, SLC7A6, AGXT2mitochondrial matrix, extracellular space, cytosolOne of 19 routes

• CHEBI:30249Reach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of lysine and tryptophan (cytosol to extracellular)
  3. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)

  Catalysed by BTD, SLC7A6, IDO1, TDO2 and 1 moremitochondrial matrix, extracellular space, cytosol

• formic acidReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of chloride and lysine (cytosol to extracellular)
  3. transport of chloride and formate (cytosol to extracellular)

  Catalysed by BTD, SLC6A14, SLC26A4mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• malonic acidReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of chloride and lysine (cytosol to extracellular)
  3. Malonate Transport, Antiport with Chloride

  Catalysed by BTD, SLC6A14mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• D-lysineReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of lysine and ornithine (cytosol to extracellular)
  3. D-lysine(in) + L-ornithine(out) = D-lysine(out) + L-ornithine(in)

  Catalysed by BTD, SLC7A5, SLC7A6, SLC25A2mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• putrescineReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of arginine and lysine (cytosol to extracellular)
  3. Putrescine Export via Putrescine/Arginine Antiporter

  Catalysed by BTD, SLC3A2, SLC7A5, SLC7A6 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 4 routes

• D-arginineReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of lysine and ornithine (cytosol to extracellular)
  3. D-arginine(in) + L-ornithine(out) = D-arginine(out) + L-ornithine(in)

  Catalysed by BTD, SLC7A5, SLC7A6, SLC25A2mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of lysine and methionine (cytosol to extracellular)
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by BTD, SLC7A6, IL4I1mitochondrial matrix, extracellular space, cytosol

• di-mu-sulfido-diiron(2+)Reach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of cysteine and lysine (cytosol to extracellular)
  3. De novo synthesis of 2Fe2S

  Catalysed by BTD, SLC7A6, NDUFAB1, FDXR and 5 moremitochondrial matrix, extracellular space, cytosol

• (S)-3-methyl-2-oxopentanoateReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of isoleucine and lysine (cytosol to extracellular)
  3. L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by BTD, SLC7A6, BCAT2, BCAT1mitochondrial matrix, extracellular space, cytosol

• D-cysteineReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of alanine and lysine (cytosol to extracellular)
  3. D-cysteine(out) + L-alanine(in) = D-cysteine(in) + L-alanine(out)

  Catalysed by BTD, SLC7A6, SLC7A10mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-serineReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of alanine and lysine (cytosol to extracellular)
  3. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)

  Catalysed by BTD, SLC7A6, SLC7A10mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 10 routes

• 5-aminolevulinateReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of glycine and lysine (cytosol to extracellular)
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by BTD, SLC7A6, ALAS1, ALAS2mitochondrial matrix, extracellular space, cytosol

• 3-methyl-2-oxovaleric acidReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of isoleucine and lysine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by BTD, SLC7A6, BCAT1mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 3-(4-hydroxyphenyl)pyruvateReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of lysine and tyrosine (cytosol to extracellular)
  3. L-tyrosine:2-oxoglutarate aminotransferase

  Catalysed by BTD, SLC7A6, GOT2, GOT1 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• glycochenodeoxycholateReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of glycine and lysine (cytosol to extracellular)
  3. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)

  Catalysed by BTD, SLC7A6, BAATmitochondrial matrix, extracellular space, cytosol

• glycochenodeoxycholic acidReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of glycine and lysine (cytosol to extracellular)
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by BTD, SLC7A6, BAATmitochondrial matrix, extracellular space, cytosol, peroxisomeOne of 3 routes

• glyoxylateReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of glycine and lysine (cytosol to extracellular)
  3. glycine + 2-oxoglutarate = glyoxylate + L-glutamate

  Catalysed by BTD, SLC7A6, AADATmitochondrial matrix, extracellular space, cytosolOne of 11 routes

• glycolithocholic acidReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of glycine and lysine (cytosol to extracellular)
  3. bile acid-CoA:amino acid N-acyltransferase (glycine)

  Catalysed by BTD, SLC7A6, BAATmitochondrial matrix, extracellular space, cytosolOne of 2 routes

• L-2-amino-3-oxobutanoic acidReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of glycine and lysine (cytosol to extracellular)
  3. Acetyl-CoA:glycine C-acetyltransferase

  Catalysed by BTD, SLC7A6, GCATmitochondrial matrix, extracellular space, cytosol

• gamma-Glu-AlaReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of alanine and lysine (cytosol to extracellular)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by BTD, SLC7A6, GGT1, GGT5 and 4 moremitochondrial matrix, extracellular space, cytosol

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of glycine and lysine (cytosol to extracellular)
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

  Catalysed by BTD, SLC7A6, GLDC, GCSHmitochondrial matrix, extracellular space, cytosol

• taurineReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. transport of chloride and lysine (cytosol to extracellular)
  3. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3

  Catalysed by BTD, SLC6A14, SLC6A6mitochondrial matrix, extracellular space, cytosolOne of 4 routes

• (R)-methylmalonyl-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by BTD, ALDH6A1mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by BTDmitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by BTD, ACP2mitochondrial matrix, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by BTDmitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by BTD, ACAA1, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by BTD, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• butanoyl-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by BTD, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 9 routes

• dodecanoyl-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by BTD, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 9 routes

• glutaryl-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. Production of Glutaryl Carnitine

  Catalysed by BTD, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

• eicosanoyl-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by BTD, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 12 routes

• itaconyl-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by BTD, SUCLG1, SUCLG2, SUCLA2mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by BTD, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 13 routes

• tetradecanoyl-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by BTD, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 11 routes

• octanoyl-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by BTD, CROTmitochondrial matrixOne of 20 routes

• phytanoyl-CoAReach from here →
  1. Biotinidase (Biotin), Mitochondrial
  2. Acetylation of Lysine
  3. phytanate-CoA ligase

  Catalysed by BTDmitochondrial matrix, cytosolOne of 5 routes