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Showing metabocard for 2-Methylacetoacetic acid (HMDB0003771)

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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2006-08-13 04:36:31 UTC
Update Date2023-02-21 17:16:49 UTC
HMDB IDHMDB0003771
Secondary Accession Numbers
  • HMDB0029172
  • HMDB03771
  • HMDB29172
Metabolite Identification
Common Name2-Methylacetoacetic acid
Description2-Methylacetoacetic acid (CAS: 2382-59-4) is a metabolite that has an increased excretion in patients with acetoacetyl-CoA thiolase (EC 2.3.1.9) deficiency (OMIM: 607809 , ACAT1; OMIM: 100678 , ACAT2; OMIM: 604770 , ACAA2; OMIM: 607809 ). Thiolases are ubiquitous and important enzymes. Several isoenzymes are known, which can occur in the cytosol (ACAT2), the mitochondria (ACAT1), or the peroxisomes (ACAA2). Thiolases are CoA-dependent enzymes which catalyze the formation of a carbon-carbon bond in a Claisen condensation step and its reverse reaction via a thiolytic degradation mechanism. Mitochondrial acetoacetyl-coenzyme A (CoA) thiolase (T2) is important in the pathways for the synthesis and degradation of ketone bodies as well as for the degradation of 2-methylacetoacetyl-CoA (PMID: 17371050 , 14518824 , 9744475 , 9001814 ). Moreover, 2-methylacetoacetic acid is found to be associated with beta-ketothiolase deficiency, which is also an inborn error of metabolism. 2-Methylacetoacetic acid is found in urine and can be used as a biomarker for the diagnosis of beta-ketothiolase deficiency.
Structure
Data?1676999809
MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0003771 (2-Methylacetoacetic acid)


  Mrv1652307191923422D          

  8  7  0  0  0  0            999 V2000
   -2.5667    0.7919    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2812    0.3794    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5667    1.6169    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8523    0.3794    0.0000 C   0  0  2  0  0  0  0  0  0  0  0  0
   -1.8523   -0.4455    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1378    0.7919    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1378    1.6169    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4233    0.3794    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  4  1  0  0  0  0
  4  5  1  1  0  0  0
  4  6  1  0  0  0  0
  6  8  1  0  0  0  0
  1  3  2  0  0  0  0
  6  7  2  0  0  0  0
  2  1  1  0  0  0  0
M  END
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3D MOL for HMDB0003771 (2-Methylacetoacetic acid)

HMDB0003771
     RDKit          3D
2-Methylacetoacetic acid
 16 15  0  0  0  0  0  0  0  0999 V2000
    2.1814    0.3323    0.2203 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.0147   -0.4758   -0.2569 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.2593   -1.6330   -0.5711 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3457    0.1143   -0.3347 C   0  0  1  0  0  0  0  0  0  0  0  0
   -1.2787   -0.9802   -0.8562 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8617    0.5123    0.9923 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1214    0.3135    1.9870 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1212    1.0803    1.0886 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.4481   -0.0405    1.2209 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.8842    1.3959    0.2062 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.0618    0.2137   -0.4255 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4118    0.9616   -1.0333 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7914   -1.3660   -1.7787 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2545   -1.7920   -0.0881 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2834   -0.6077   -1.0616 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3797    1.9713    0.6908 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  2  0
  2  4  1  0
  4  5  1  0
  4  6  1  0
  6  7  2  0
  6  8  1  0
  1  9  1  0
  1 10  1  0
  1 11  1  0
  4 12  1  6
  5 13  1  0
  5 14  1  0
  5 15  1  0
  8 16  1  0
M  END
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3D SDF for HMDB0003771 (2-Methylacetoacetic acid)


  Mrv1652307191923422D          

  8  7  0  0  0  0            999 V2000
   -2.5667    0.7919    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2812    0.3794    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5667    1.6169    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8523    0.3794    0.0000 C   0  0  2  0  0  0  0  0  0  0  0  0
   -1.8523   -0.4455    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1378    0.7919    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1378    1.6169    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4233    0.3794    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  4  1  0  0  0  0
  4  5  1  1  0  0  0
  4  6  1  0  0  0  0
  6  8  1  0  0  0  0
  1  3  2  0  0  0  0
  6  7  2  0  0  0  0
  2  1  1  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0003771

> <DATABASE_NAME>
hmdb

> <SMILES>
C[C@H](C(C)=O)C(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C5H8O3/c1-3(4(2)6)5(7)8/h3H,1-2H3,(H,7,8)/t3-/m1/s1

> <INCHI_KEY>
GCXJINGJZAOJHR-GSVOUGTGSA-N

> <FORMULA>
C5H8O3

> <MOLECULAR_WEIGHT>
116.116

> <EXACT_MASS>
116.047344118

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
16

> <JCHEM_AVERAGE_POLARIZABILITY>
11.025900997427922

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
1

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
(2R)-2-methyl-3-oxobutanoic acid

> <ALOGPS_LOGP>
0.20

> <JCHEM_LOGP>
0.5414945326666666

> <ALOGPS_LOGS>
-0.09

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA>
15.762517895774014

> <JCHEM_PKA_STRONGEST_ACIDIC>
4.174423632224772

> <JCHEM_PKA_STRONGEST_BASIC>
-7.532727734312502

> <JCHEM_POLAR_SURFACE_AREA>
54.37

> <JCHEM_REFRACTIVITY>
27.113400000000006

> <JCHEM_ROTATABLE_BOND_COUNT>
2

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
9.49e+01 g/l

> <JCHEM_TRADITIONAL_IUPAC>
(2R)-2-methyl-3-oxobutanoic acid

> <JCHEM_VEBER_RULE>
0

$$$$
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3D-SDF for HMDB0003771 (2-Methylacetoacetic acid)

HMDB0003771
     RDKit          3D
2-Methylacetoacetic acid
 16 15  0  0  0  0  0  0  0  0999 V2000
    2.1814    0.3323    0.2203 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.0147   -0.4758   -0.2569 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.2593   -1.6330   -0.5711 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3457    0.1143   -0.3347 C   0  0  1  0  0  0  0  0  0  0  0  0
   -1.2787   -0.9802   -0.8562 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8617    0.5123    0.9923 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1214    0.3135    1.9870 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1212    1.0803    1.0886 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.4481   -0.0405    1.2209 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.8842    1.3959    0.2062 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.0618    0.2137   -0.4255 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4118    0.9616   -1.0333 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7914   -1.3660   -1.7787 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2545   -1.7920   -0.0881 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2834   -0.6077   -1.0616 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3797    1.9713    0.6908 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  2  0
  2  4  1  0
  4  5  1  0
  4  6  1  0
  6  7  2  0
  6  8  1  0
  1  9  1  0
  1 10  1  0
  1 11  1  0
  4 12  1  6
  5 13  1  0
  5 14  1  0
  5 15  1  0
  8 16  1  0
M  END
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PDB for HMDB0003771 (2-Methylacetoacetic acid)

HEADER    PROTEIN                                 19-JUL-19   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   19-JUL-19         0                                  
HETATM    1  C   UNK     0      -4.791   1.478   0.000  0.00  0.00           C+0
HETATM    2  O   UNK     0      -6.125   0.708   0.000  0.00  0.00           O+0
HETATM    3  O   UNK     0      -4.791   3.018   0.000  0.00  0.00           O+0
HETATM    4  C   UNK     0      -3.458   0.708   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0      -3.458  -0.832   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0      -2.124   1.478   0.000  0.00  0.00           C+0
HETATM    7  O   UNK     0      -2.124   3.018   0.000  0.00  0.00           O+0
HETATM    8  C   UNK     0      -0.790   0.708   0.000  0.00  0.00           C+0
CONECT    1    4    3    2                                                  
CONECT    2    1                                                            
CONECT    3    1                                                            
CONECT    4    1    5    6                                                  
CONECT    5    4                                                            
CONECT    6    4    8    7                                                  
CONECT    7    6                                                            
CONECT    8    6                                                            
MASTER        0    0    0    0    0    0    0    0    8    0   14    0
END
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3D PDB for HMDB0003771 (2-Methylacetoacetic acid)

COMPND    HMDB0003771
HETATM    1  C1  UNL     1       2.181   0.332   0.220  1.00  0.00           C  
HETATM    2  C2  UNL     1       1.015  -0.476  -0.257  1.00  0.00           C  
HETATM    3  O1  UNL     1       1.259  -1.633  -0.571  1.00  0.00           O  
HETATM    4  C3  UNL     1      -0.346   0.114  -0.335  1.00  0.00           C  
HETATM    5  C4  UNL     1      -1.279  -0.980  -0.856  1.00  0.00           C  
HETATM    6  C5  UNL     1      -0.862   0.512   0.992  1.00  0.00           C  
HETATM    7  O2  UNL     1      -0.121   0.313   1.987  1.00  0.00           O  
HETATM    8  O3  UNL     1      -2.121   1.080   1.089  1.00  0.00           O  
HETATM    9  H1  UNL     1       2.448  -0.041   1.221  1.00  0.00           H  
HETATM   10  H2  UNL     1       1.884   1.396   0.206  1.00  0.00           H  
HETATM   11  H3  UNL     1       3.062   0.214  -0.425  1.00  0.00           H  
HETATM   12  H4  UNL     1      -0.412   0.962  -1.033  1.00  0.00           H  
HETATM   13  H5  UNL     1      -0.791  -1.366  -1.779  1.00  0.00           H  
HETATM   14  H6  UNL     1      -1.255  -1.792  -0.088  1.00  0.00           H  
HETATM   15  H7  UNL     1      -2.283  -0.608  -1.062  1.00  0.00           H  
HETATM   16  H8  UNL     1      -2.380   1.971   0.691  1.00  0.00           H  
CONECT    1    2    9   10   11
CONECT    2    3    3    4
CONECT    4    5    6   12
CONECT    5   13   14   15
CONECT    6    7    7    8
CONECT    8   16
END
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SMILES for HMDB0003771 (2-Methylacetoacetic acid)

C[C@H](C(C)=O)C(O)=O
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INCHI for HMDB0003771 (2-Methylacetoacetic acid)

InChI=1S/C5H8O3/c1-3(4(2)6)5(7)8/h3H,1-2H3,(H,7,8)/t3-/m1/s1
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View in JSmolView NMR AssignmentsView Stereo Labels
Synonyms
ValueSource
2-MethylacetoacetateGenerator
2-Methyl-3-oxo-butyric acidHMDB
2-Methyl-3-oxo-butyrateHMDB
2Methyl-3-ketovalerateHMDB
(+/-)-2-methyl-3-oxobutanoateHMDB
(+/-)-2-methyl-3-oxobutanoic acidHMDB
2-Acetylpropionic acidHMDB
2-Methyl-3-ketobutyric acidHMDB
2-Methyl-3-oxo-butanoateHMDB
2-Methyl-3-oxo-butanoic acidHMDB
2-Methyl-3-oxobutanoateHMDB
2-Methyl-3-oxobutanoic acidHMDB
2-Methyl-3-oxobutyric acidHMDB
2-Methyl-acetoacetic acidHMDB
3-oxo-2-Methylbutyric acidHMDB
a-Methylacetoacetic acidHMDB
alpha-MethylacetoacetateHMDB
alpha-Methylacetoacetic acidHMDB
(-)-2-Methyl-3-oxobutanoic acidHMDB
(2S)-2-Methyl-3-oxobutanoic acidHMDB
(S)-2-Acetylpropionic acidHMDB
(S)-2-Methyl-3-oxobutyric acidHMDB
(±)-2-methyl-3-oxobutanoic acidHMDB
3-Keto-2-methylbutyrateHMDB
3-Keto-2-methylbutyric acidHMDB
Α-methylacetoacetic acidHMDB
2-Methylacetoacetic acidHMDB
Chemical FormulaC5H8O3
Average Molecular Weight116.116
Monoisotopic Molecular Weight116.047344118
IUPAC Name(2R)-2-methyl-3-oxobutanoic acid
Traditional Name(2R)-2-methyl-3-oxobutanoic acid
CAS Registry Number76652-88-5
SMILES
C[C@H](C(C)=O)C(O)=O
InChI Identifier
InChI=1S/C5H8O3/c1-3(4(2)6)5(7)8/h3H,1-2H3,(H,7,8)/t3-/m1/s1
InChI KeyGCXJINGJZAOJHR-GSVOUGTGSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassKeto acids and derivatives
Sub ClassShort-chain keto acids and derivatives
Direct ParentShort-chain keto acids and derivatives
Alternative Parents
Substituents
  • Beta-keto acid
  • Branched fatty acid
  • Methyl-branched fatty acid
  • Short-chain keto acid
  • Beta-hydroxy ketone
  • Fatty acyl
  • 1,3-dicarbonyl compound
  • Ketone
  • Carboxylic acid derivative
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Carbonyl group
  • Hydrocarbon derivative
  • Organic oxygen compound
  • Organooxygen compound
  • Organic oxide
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
Physiological effectNot Available
Disposition
Biological locationSource
Process
Naturally occurring process
Role
Biological role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility94.9 g/LALOGPS
logP0.2ALOGPS
logP0.54ChemAxon
logS-0.09ALOGPS
pKa (Strongest Acidic)4.17ChemAxon
pKa (Strongest Basic)-7.5ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity27.11 m³·mol⁻¹ChemAxon
Polarizability11.03 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DeepCCS[M+H]+121.06630932474
DeepCCS[M-H]-117.39830932474
DeepCCS[M-2H]-154.65230932474
DeepCCS[M+Na]+129.72930932474

Predicted Retention Times

Underivatized

Chromatographic MethodRetention TimeReference
Measured using a Waters Acquity ultraperformance liquid chromatography (UPLC) ethylene-bridged hybrid (BEH) C18 column (100 mm × 2.1 mm; 1.7 μmparticle diameter). Predicted by Afia on May 17, 2022. Predicted by Afia on May 17, 2022.2.67 minutes32390414
Predicted by Siyang on May 30, 20229.9718 minutes33406817
Predicted by Siyang using ReTip algorithm on June 8, 20222.7 minutes32390414
AjsUoB = Accucore 150 Amide HILIC with 10mM Ammonium Formate, 0.1% Formic Acid85.8 seconds40023050
Fem_Long = Waters ACQUITY UPLC HSS T3 C18 with Water:MeOH and 0.1% Formic Acid1207.3 seconds40023050
Fem_Lipids = Ascentis Express C18 with (60:40 water:ACN):(90:10 IPA:ACN) and 10mM NH4COOH + 0.1% Formic Acid357.8 seconds40023050
Life_Old = Waters ACQUITY UPLC BEH C18 with Water:(20:80 acetone:ACN) and 0.1% Formic Acid92.5 seconds40023050
Life_New = RP Waters ACQUITY UPLC HSS T3 C18 with Water:(30:70 MeOH:ACN) and 0.1% Formic Acid220.1 seconds40023050
RIKEN = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid60.0 seconds40023050
Eawag_XBridgeC18 = XBridge C18 3.5u 2.1x50 mm with Water:MeOH and 0.1% Formic Acid305.7 seconds40023050
BfG_NTS_RP1 =Agilent Zorbax Eclipse Plus C18 (2.1 mm x 150 mm, 3.5 um) with Water:ACN and 0.1% Formic Acid342.0 seconds40023050
HILIC_BDD_2 = Merck SeQuant ZIC-HILIC with ACN(0.1% formic acid):water(16 mM ammonium formate)111.5 seconds40023050
UniToyama_Atlantis = RP Waters Atlantis T3 (2.1 x 150 mm, 5 um) with ACN:Water and 0.1% Formic Acid697.5 seconds40023050
BDD_C18 = Hypersil Gold 1.9µm C18 with Water:ACN and 0.1% Formic Acid263.8 seconds40023050
UFZ_Phenomenex = Kinetex Core-Shell C18 2.6 um, 3.0 x 100 mm, Phenomenex with Water:MeOH and 0.1% Formic Acid915.0 seconds40023050
SNU_RIKEN_POS = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid232.2 seconds40023050
RPMMFDA = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid271.3 seconds40023050
MTBLS87 = Merck SeQuant ZIC-pHILIC column with ACN:Water and :ammonium carbonate482.5 seconds40023050
KI_GIAR_zic_HILIC_pH2_7 = Merck SeQuant ZIC-HILIC with ACN:Water and 0.1% FA295.9 seconds40023050
Meister zic-pHILIC pH9.3 = Merck SeQuant ZIC-pHILIC column with ACN:Water 5mM NH4Ac pH9.3 and 5mM ammonium acetate in water168.1 seconds40023050

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
2-Methylacetoacetic acidC[C@H](C(C)=O)C(O)=O1885.2Standard polar33892256
2-Methylacetoacetic acidC[C@H](C(C)=O)C(O)=O917.9Standard non polar33892256
2-Methylacetoacetic acidC[C@H](C(C)=O)C(O)=O1019.3Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
2-Methylacetoacetic acid,1TMS,isomer #1CC(=O)[C@@H](C)C(=O)O[Si](C)(C)C1079.1Semi standard non polar33892256
2-Methylacetoacetic acid,1TMS,isomer #2CC(O[Si](C)(C)C)=C(C)C(=O)O1183.9Semi standard non polar33892256
2-Methylacetoacetic acid,1TMS,isomer #3C=C(O[Si](C)(C)C)[C@@H](C)C(=O)O1152.7Semi standard non polar33892256
2-Methylacetoacetic acid,2TMS,isomer #1CC(O[Si](C)(C)C)=C(C)C(=O)O[Si](C)(C)C1302.1Semi standard non polar33892256
2-Methylacetoacetic acid,2TMS,isomer #1CC(O[Si](C)(C)C)=C(C)C(=O)O[Si](C)(C)C1269.3Standard non polar33892256
2-Methylacetoacetic acid,2TMS,isomer #1CC(O[Si](C)(C)C)=C(C)C(=O)O[Si](C)(C)C1263.4Standard polar33892256
2-Methylacetoacetic acid,2TMS,isomer #2C=C(O[Si](C)(C)C)[C@@H](C)C(=O)O[Si](C)(C)C1208.4Semi standard non polar33892256
2-Methylacetoacetic acid,2TMS,isomer #2C=C(O[Si](C)(C)C)[C@@H](C)C(=O)O[Si](C)(C)C1209.5Standard non polar33892256
2-Methylacetoacetic acid,2TMS,isomer #2C=C(O[Si](C)(C)C)[C@@H](C)C(=O)O[Si](C)(C)C1295.7Standard polar33892256
2-Methylacetoacetic acid,1TBDMS,isomer #1CC(=O)[C@@H](C)C(=O)O[Si](C)(C)C(C)(C)C1286.2Semi standard non polar33892256
2-Methylacetoacetic acid,1TBDMS,isomer #2CC(O[Si](C)(C)C(C)(C)C)=C(C)C(=O)O1412.2Semi standard non polar33892256
2-Methylacetoacetic acid,1TBDMS,isomer #3C=C(O[Si](C)(C)C(C)(C)C)[C@@H](C)C(=O)O1355.6Semi standard non polar33892256
2-Methylacetoacetic acid,2TBDMS,isomer #1CC(O[Si](C)(C)C(C)(C)C)=C(C)C(=O)O[Si](C)(C)C(C)(C)C1693.1Semi standard non polar33892256
2-Methylacetoacetic acid,2TBDMS,isomer #1CC(O[Si](C)(C)C(C)(C)C)=C(C)C(=O)O[Si](C)(C)C(C)(C)C1701.8Standard non polar33892256
2-Methylacetoacetic acid,2TBDMS,isomer #1CC(O[Si](C)(C)C(C)(C)C)=C(C)C(=O)O[Si](C)(C)C(C)(C)C1600.6Standard polar33892256
2-Methylacetoacetic acid,2TBDMS,isomer #2C=C(O[Si](C)(C)C(C)(C)C)[C@@H](C)C(=O)O[Si](C)(C)C(C)(C)C1637.9Semi standard non polar33892256
2-Methylacetoacetic acid,2TBDMS,isomer #2C=C(O[Si](C)(C)C(C)(C)C)[C@@H](C)C(=O)O[Si](C)(C)C(C)(C)C1650.7Standard non polar33892256
2-Methylacetoacetic acid,2TBDMS,isomer #2C=C(O[Si](C)(C)C(C)(C)C)[C@@H](C)C(=O)O[Si](C)(C)C(C)(C)C1611.6Standard polar33892256
Spectra
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodExpected but not QuantifiedNot QuantifiedNot AvailableNot Available
Normal
      Not Available
 details
UrineDetected and Quantified<2 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
 details
UrineDetected and Quantified0 umol/mmol creatinineAdult (>18 years old)Both
Normal		 details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified325.0 (0.0-650.0) umol/mmol creatinineChildren (1-13 years old)BothBeta-Ketothiolase deficiency
    • MetaGene: Metabol...
 details
UrineDetected and Quantified8030 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedBeta-ketothiolase deficiency details
UrineDetected and Quantified3368 umol/mmol creatinineChildren (1-13 years old)Not Specified
Beta-ketothiolase deficiency		 details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothBladder cancer details
Associated Disorders and Diseases
Disease References
Beta-ketothiolase deficiency
  1. Fontaine M, Briand G, Ser N, Armelin I, Rolland MO, Degand P, Vamecq J: Metabolic studies in twin brothers with 2-methylacetoacetyl-CoA thiolase deficiency. Clin Chim Acta. 1996 Nov 15;255(1):67-83. [PubMed:8930414 ]
  2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Associated OMIM IDs
  • 203750 (Beta-ketothiolase deficiency)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB023226
KNApSAcK IDNot Available
Chemspider ID24191898
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound40578931
PDB IDNot Available
ChEBI ID37079
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDNot Available
Good Scents IDNot Available
References
Synthesis ReferenceMiddleton B; Bartlett K The synthesis and characterisation of 2-methylacetoacetyl coenzyme A and its use in the identification of the site of the defect in 2-methylacetoacetic and 2-methyl-3-hydroxybutyric aciduria. Clinica chimica acta; international journal of clinical chemistry (1983), 128(2-3), 291-305.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Haapalainen AM, Merilainen G, Pirila PL, Kondo N, Fukao T, Wierenga RK: Crystallographic and kinetic studies of human mitochondrial acetoacetyl-CoA thiolase: the importance of potassium and chloride ions for its structure and function. Biochemistry. 2007 Apr 10;46(14):4305-21. Epub 2007 Mar 20. [PubMed:17371050 ]
  2. Fukao T, Nakamura H, Song XQ, Nakamura K, Orii KE, Kohno Y, Kano M, Yamaguchi S, Hashimoto T, Orii T, Kondo N: Characterization of N93S, I312T, and A333P missense mutations in two Japanese families with mitochondrial acetoacetyl-CoA thiolase deficiency. Hum Mutat. 1998;12(4):245-54. [PubMed:9744475 ]
  3. Fukao T, Zhang GX, Sakura N, Kubo T, Yamaga H, Hazama A, Kohno Y, Matsuo N, Kondo M, Yamaguchi S, Shigematsu Y, Kondo N: The mitochondrial acetoacetyl-CoA thiolase (T2) deficiency in Japanese patients: urinary organic acid and blood acylcarnitine profiles under stable conditions have subtle abnormalities in T2-deficient patients with some residual T2 activity. J Inherit Metab Dis. 2003;26(5):423-31. [PubMed:14518824 ]
  4. Fukao T, Kodama A, Aoyanagi N, Tsukino R, Uemura S, Song XQ, Watanebe H, Kuhara T, Matsumoto I, Orii T, Kondo N: Mild form of beta-ketothiolase deficiency (mitochondrial acetoacetyl-CoA thiolase deficiency) in two Japanese siblings: identification of detectable residual activity and cross-reactive material in EB-transformed lymphocytes. Clin Genet. 1996 Oct;50(4):263-6. [PubMed:9001814 ]