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Reach

(R)-3-aminoisobutyric acid

362,296 routes · scope human · up to 3 steps · built 5 October 2026 · Reactions · API

Inferred

Every route here is a hypothesis drawn from reference reactions (Rhea and Human-GEM), not a finding. Currency species such as water, ATP and NAD(H) never form a step. Evidence key

430 compounds within 3 steps: 2 in 1, 52 in 2, 376 in 3

Does it reach a compound?

Two steps

L-prolineReach from here →
1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
2. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)
Catalysed by AGXT2, SLC1A4mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes
L-cysteinylglycineReach from here →
1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
2. gamma-glutamyltransferase (alanine)
Catalysed by AGXT2, GGTLC3, GGT1, GGT5 and 3 moremitochondrial matrix, cytosolOne of 2 routes
Mo-molybdopterinReach from here →
1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
Catalysed by AGXT2, MOCOSmitochondrial matrix
(2S)-2-aminobutanoateReach from here →
1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
Catalysed by AGXT2mitochondrial matrix

Three steps

beta-carbolineReach from here →
Catalysed by AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosol
(S)-carnitineReach from here →
Catalysed by ABAT,

Page 2 of 9

AGXT2

, ALDH6A1

mitochondrial matrix, cytosol

One of 247 routes

2-hydroxybutyric acidReach from here →

(S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)

Catalysed by ABAT, AGXT2, SLC16A4, SLC16A5 and 4 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

3-methyl-2-oxobutanoateReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
transport of alanine and valine (cytosol to extracellular)
L-Valine:2-oxoglutarate aminotransferase

Catalysed by AGXT2, SLC7A5, BCAT2mitochondrial matrix, extracellular space, cytosolOne of 3 routes

(8S)-3',8-cyclo-7,8-dihydroguanosine 5'-triphosphateReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
GTP + AH2 + S-adenosyl-L-methionine = (8S)-3',8-cyclo-7,8-dihydroguanosine 5'-triphosphate + 5'-deoxyadenosine + L-methionine + A + H(+)

Catalysed by AGXT2, MOCOS, MOCS1mitochondrial matrix

AReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
transport of alanine and cysteine (cytosol to extracellular)
Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

Catalysed by AGXT2, SLC7A5, MOCOSmitochondrial matrix, extracellular space, cytosolOne of 72 routes

glycoursodeoxycholateReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
L-Alanine:glyoxylate aminotransferase
glycine to glycoursodeoxycholate conversion

Catalysed by AGXT2, AGXTmitochondrial matrix, cytosolOne of 2 routes

(18S)-hydroxy-(5Z,8Z,11Z,14Z,16E)-eicosapentaenoateReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
(5Z,8Z,11Z,14Z,17Z)-eicosapentaenoate + AH2 + O2 = (18S)-hydroxy-(5Z,8Z,11Z,14Z,16E)-eicosapentaenoate + A + H2O

Catalysed by AGXT2, MOCOS, PTGS2mitochondrial matrix

(15S)-hydroxy-(5Z,8Z,11Z,13E,17Z)-eicosapentaenoateReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
(5Z,8Z,11Z,14Z,17Z)-eicosapentaenoate + AH2 + O2 = (15S)-hydroxy-(5Z,8Z,11Z,13E,17Z)-eicosapentaenoate + A + H2O

Catalysed by AGXT2, MOCOS, PTGS2mitochondrial matrix

prostaglandin H3Reach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
prostaglandin G3 + AH2 = prostaglandin H3 + A + H2O

Catalysed by AGXT2, MOCOS, PTGS2mitochondrial matrix

13-hydroxy-(9Z,11E)-octadecadienoateReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
(9Z,12Z)-octadecadienoate + AH2 + O2 = 13-hydroxy-(9Z,11E)-octadecadienoate + A + H2O

Catalysed by AGXT2, MOCOS, PTGS2mitochondrial matrix

9-hydroxy-(10E,12Z)-octadecadienoateReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
(9Z,12Z)-octadecadienoate + AH2 + O2 = 9-hydroxy-(10E,12Z)-octadecadienoate + A + H2O

Catalysed by AGXT2, MOCOS, PTGS2mitochondrial matrix

N-(9Z-octadecenoyl)glycineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
L-Alanine:glyoxylate aminotransferase
(9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)

Catalysed by AGXT2, AGXT, GLYATL3, GLYATL2mitochondrial matrix

N-(9Z-octadecenoyl)-L-phenylalanineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
transport of alanine and phenylalanine (cytosol to extracellular)
L-phenylalanine + (9Z)-octadecenoate = N-(9Z-octadecenoyl)-L-phenylalanine + H2O

Catalysed by AGXT2, SLC7A5, PM20D1mitochondrial matrix, extracellular space, cytosol

N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-L-phenylalanineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
transport of alanine and phenylalanine (cytosol to extracellular)
(5Z,8Z,11Z,14Z)-eicosatetraenoate + L-phenylalanine = N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-L-phenylalanine + H2O

Catalysed by AGXT2, SLC7A5, PM20D1mitochondrial matrix, extracellular space, cytosol

N-(9Z-octadecenoyl)-L-leucineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
transport of alanine and leucine (cytosol to extracellular)
N-(9Z-octadecenoyl)-L-leucine + H2O = L-leucine + (9Z)-octadecenoate

Catalysed by AGXT2, SLC7A5, PM20D1mitochondrial matrix, extracellular space, cytosol

N-octadecanoylglycineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
L-Alanine:glyoxylate aminotransferase
octadecanoyl-CoA + glycine = N-octadecanoylglycine + CoA + H(+)

Catalysed by AGXT2, AGXT, GLYATL2mitochondrial matrix

N-(9Z-hexadecenoyl)-glycineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
L-Alanine:glyoxylate aminotransferase
(9Z)-hexadecenoyl-CoA + glycine = N-(9Z-hexadecenoyl)-glycine + CoA + H(+)

Catalysed by AGXT2, AGXT, GLYATL2mitochondrial matrix

(13R)-hydroxy-(9Z,11E)-octadecadienoateReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
(9Z,12Z)-octadecadienoate + AH2 + O2 = (13R)-hydroxy-(9Z,11E)-octadecadienoate + A + H2O

Catalysed by AGXT2, MOCOS, PTGS1, PTGS2mitochondrial matrix

acetoacetateReach from here →

(S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
transport of acetoacetate and pyruvate (cytosol to mitochondria)

Catalysed by ABAT, AGXT2mitochondrial matrix, cytosolOne of 4 routes

2-[(11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoyl]-sn-glycero-3-phosphocholineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphocholine + AH2 + O2 = 2-[(11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoyl]-sn-glycero-3-phosphocholine + A + H2O

Catalysed by AGXT2, MOCOS, PTGS2mitochondrial matrix

2-[(15R)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoyl]-sn-glycero-3-phosphocholineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphocholine + AH2 + O2 = 2-[(15R)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoyl]-sn-glycero-3-phosphocholine + A + H2O

Catalysed by AGXT2, MOCOS, PTGS2mitochondrial matrix

2-[(15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoyl]-sn-glycero-3-phosphocholineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphocholine + AH2 + O2 = 2-[(15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoyl]-sn-glycero-3-phosphocholine + A + H2O

Catalysed by AGXT2, MOCOS, PTGS2mitochondrial matrix

2-(prostaglandin H2)-sn-glycero-3-phosphoethanolamineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
2-(prostaglandin G2)-sn-glycero-3-phosphoethanolamine + AH2 = 2-(prostaglandin H2)-sn-glycero-3-phosphoethanolamine + A + H2O

Catalysed by AGXT2, MOCOS, PTGS2mitochondrial matrix

2-(prostaglandin H2)-sn-glycero-3-phosphocholineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
2-(prostaglandin G2)-sn-glycero-3-phosphocholine + AH2 = 2-(prostaglandin H2)-sn-glycero-3-phosphocholine + A + H2O

Catalysed by AGXT2, MOCOS, PTGS2mitochondrial matrix

N-(4-oxoglutaryl)-L-cysteinylglycineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
gamma-glutamyltransferase (alanine)
N-(4-oxoglutaryl)-L-cysteinylglycine + H2O = L-cysteinylglycine + 2-oxoglutarate

Catalysed by AGXT2, GGTLC3, GGT1, GGT5 and 4 moremitochondrial matrix, cytosol

N-hexadecanoylglycineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
L-Alanine:glyoxylate aminotransferase
hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)

Catalysed by AGXT2, AGXT, GLYATL3mitochondrial matrix

N-dodecanoylglycineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
L-Alanine:glyoxylate aminotransferase
dodecanoyl-CoA + glycine = N-dodecanoylglycine + CoA + H(+)

Catalysed by AGXT2, AGXT, GLYATL2mitochondrial matrix

N-decanoylglycineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
L-Alanine:glyoxylate aminotransferase
decanoyl-CoA + glycine = N-decanoylglycine + CoA + H(+)

Catalysed by AGXT2, AGXT, GLYATL2mitochondrial matrix

N-octanoylglycineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
L-Alanine:glyoxylate aminotransferase
octanoyl-CoA + glycine = N-octanoylglycine + CoA + H(+)

Catalysed by AGXT2, AGXT, GLYATL2mitochondrial matrix

D-histidineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
transport of alanine and ornithine (cytosol to extracellular)
D-histidine(in) + L-ornithine(out) + H(+)(in) = D-histidine(out) + L-ornithine(in) + H(+)(out)

Catalysed by AGXT2, SLC7A6, SLC25A2mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

L-homoarginineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
transport of alanine and arginine (cytosol to extracellular)
L-homoarginine(in) + L-arginine(out) = L-homoarginine(out) + L-arginine(in)

Catalysed by AGXT2, SLC7A6, SLC25A29mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

L-homocarnosineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
transport of alanine and histidine (cytosol to extracellular)
Formation of homocarnosine

Catalysed by AGXT2, SLC7A6, CARNS1mitochondrial matrix, extracellular space, cytosol

N(1)-(5-phospho-beta-D-ribosyl)glycinamideReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
L-Alanine:glyoxylate aminotransferase
5-phospho-beta-D-ribosylamine + glycine + ATP = N(1)-(5-phospho-beta-D-ribosyl)glycinamide + ADP + phosphate + H(+)

Catalysed by AGXT2, AGXT, GARTmitochondrial matrix

L-cystine-bis-glycineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
L-Alanine:glyoxylate aminotransferase
L-cystine-bis-glycine + 2 H2O = L-cystine + 2 glycine

Catalysed by AGXT2, AGXT, DPEP1, DPEP2mitochondrial matrixOne of 3 routes

glycyldehydrophenylalanineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
L-Alanine:glyoxylate aminotransferase
glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine

Catalysed by AGXT2, AGXT, DPEP1mitochondrial matrix

2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
transport of alanine and glutamine (cytosol to extracellular)
N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + L-glutamine + ATP + H2O = 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidine + L-glutamate + ADP + phosphate + H(+)

Catalysed by AGXT2, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosol

kinetensinReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
transport of alanine and phenylalanine (cytosol to extracellular)
Chymase

Catalysed by AGXT2, SLC7A5, CMA1mitochondrial matrix, extracellular space, cytosolOne of 3 routes

an N-(fatty acyl)-glycineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
L-Alanine:glyoxylate aminotransferase
a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

Catalysed by AGXT2, AGXT, GLYATL2mitochondrial matrix

N-(9Z,12Z-octadecadienoyl)-glycineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
L-Alanine:glyoxylate aminotransferase
(9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

Catalysed by AGXT2, AGXT, GLYATL2mitochondrial matrix

(7Z)-octadecenoyl-CoAReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
amino-acid N-acetyltransferase (N-acetyl-L-alanine)
carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)

Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes

acetaldehydeReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
transport of alanine and threonine (cytosol to extracellular)
L-threonine acetaldehyde-lyase (glycine-forming)

Catalysed by AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 2 routes

CHEBI:15345Reach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
amino-acid N-acetyltransferase (N-acetyl-L-alanine)
Acetoacetate:CoA ligase (AMP-forming)

Catalysed by AGXT2, AACSmitochondrial matrix, cytosolOne of 2 routes

cholineReach from here →

(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
amino-acid N-acetyltransferase (N-acetyl-L-alanine)
Acetyl-CoA:choline O-acetyltransferase

Catalysed by AGXT2, CHATmitochondrial matrix, nucleusOne of 2 routes

acetylpyruvateReach from here →

(S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
acetylpyruvate + H2O = acetate + pyruvate + H(+)

Catalysed by ABAT, AGXT2, FAHD1mitochondrial matrix

all-trans-retinoic acidReach from here →

(S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
(R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
transport of pyruvate and retinoate (cytosol to extracellular)

Catalysed by ABAT, AGXT2, SLC16A4, SLC16A5 and 4 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes