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Showing NP-Card for 2-Methylbutyrylglycine (NP0000232)

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Record Information
Version1.0
Created at2005-11-16 15:48:42 UTC
Updated at2020-11-24 22:13:53 UTC
NP-MRD IDNP0000232
Secondary Accession NumbersNone
Natural Product Identification
Common Name2-Methylbutyrylglycine
Description2-Methylbutyrylglycine is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism, such as propionic acidemia. In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. Acyl glycines are produced through the action of glycine N-acyltransferase (EC 2.3.1.13) Which is an enzyme that catalyzes the chemical reaction: Acyl-CoA + glycine < -- > CoA + N-acylglycine. The isolated excretion of high levels of 2-methylbutyrylglycine (2-MBG) is the hallmark of short/branched-chain acyl-CoA dehydrogenase deficiency or SBCADD (PMID:15615815 ). The disorder is also called 2-methylbutyryl-CoA dehydrogenase deficiency (PMID: 17883863 ) And has been associated with autism and mental retardation. SBCADD is a recently described autosomal recessive disorder caused by a defect in the degradation pathway of L- isoleucine leading to increased urinary excretion of 2-methylbutyryl glycine. The enzymatic defect results from disruption of the SBCAD gene. Deficiency of SBCAD leads to accumulation of its substrate, 2-methylbutyryl-CoA within the mitochondrion. This substance is transesterified with glycine by the mitochondrial enzyme acyl-CoA glycine-N-acyltransferase (glycine-N-acylase) to form 2-methylbutyryl glycine. Affected patients can be divided into two categories. The first category consists of infants detected by newborn screening programmes. These infants are treated with diet and remain without clinical symptoms. In the second category affected patients are diagnosed because they presented clinically with seizures and psychomotor delay and have increased urinary excretion of 2-methylbutyryl glycine (PMID: 17883863 ). 2-Methylbutyrylglycine has also been found in the urine of patients with propionyl-CoA carboxylase deficiency after consuming isoleucine. (PMID: 630060 ). 2-Methylbutyrylglycine is also elevated in the urine of patients with glutaric aciduria II and ethylmalonic encephalopathy.
Structure
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MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for NP0000232 (2-Methylbutyrylglycine)

2-Methylbutyrylglycine.mol
  Mrv1652305261923542D          

 11 10  0  0  0  0            999 V2000
    1.4289    0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.0000    0.2063    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    0.7145   -0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.4289    1.0313    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.1434   -0.2062    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7145   -0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7145   -1.0313    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4289    0.2062    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1434   -0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1434   -1.0313    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4289    1.0312    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  2  3  1  0  0  0  0
  1  3  1  0  0  0  0
  1  4  2  0  0  0  0
  1  5  1  0  0  0  0
  6  7  2  0  0  0  0
  6  8  1  0  0  0  0
  8  9  1  0  0  0  0
  9 10  1  0  0  0  0
  8 11  1  0  0  0  0
  2  6  1  0  0  0  0
M  END
Download

3D MOL for NP0000232 (2-Methylbutyrylglycine)

HMDB0000339
     RDKit          3D
2-Methylbutyrylglycine
 24 23  0  0  0  0  0  0  0  0999 V2000
   -3.3634    0.4052    0.6312 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9082    0.2303    0.9676 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1226   -0.3679   -0.1729 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1714    0.4815   -1.4121 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3071   -0.4402    0.2637 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.6237   -0.0259    1.4237 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.3021   -0.9617   -0.5971 N   0  0  0  0  0  0  0  0  0  0  0  0
    2.6615   -1.0068   -0.1243 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.1210    0.3608    0.1950 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.3873    1.3660    0.0516 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.4404    0.4742    0.6738 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5215    1.3264    0.0678 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6955   -0.4815    0.0779 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.9120    0.4198    1.6168 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4281    1.1712    1.3061 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8269   -0.5053    1.8165 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4609   -1.4097   -0.3627 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0217    0.2254   -2.0761 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2296    0.2920   -1.9741 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2069    1.5451   -1.0984 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.0069   -1.2886   -1.5315 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.7165   -1.6222    0.7962 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.3523   -1.4776   -0.8508 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.9496    1.2897    0.3122 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  1  0
  3  5  1  0
  5  6  2  0
  5  7  1  0
  7  8  1  0
  8  9  1  0
  9 10  2  0
  9 11  1  0
  1 12  1  0
  1 13  1  0
  1 14  1  0
  2 15  1  0
  2 16  1  0
  3 17  1  0
  4 18  1  0
  4 19  1  0
  4 20  1  0
  7 21  1  0
  8 22  1  0
  8 23  1  0
 11 24  1  0
M  END
Download

3D SDF for NP0000232 (2-Methylbutyrylglycine)

2-Methylbutyrylglycine.mol
  Mrv1652305261923542D          

 11 10  0  0  0  0            999 V2000
    1.4289    0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.0000    0.2063    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    0.7145   -0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.4289    1.0313    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.1434   -0.2062    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7145   -0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7145   -1.0313    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4289    0.2062    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1434   -0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1434   -1.0313    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4289    1.0312    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  2  3  1  0  0  0  0
  1  3  1  0  0  0  0
  1  4  2  0  0  0  0
  1  5  1  0  0  0  0
  6  7  2  0  0  0  0
  6  8  1  0  0  0  0
  8  9  1  0  0  0  0
  9 10  1  0  0  0  0
  8 11  1  0  0  0  0
  2  6  1  0  0  0  0
M  END
> <DATABASE_ID>
NP0000232

> <DATABASE_NAME>
NP-MRD

> <SMILES>
CCC(C)C(=O)NCC(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C7H13NO3/c1-3-5(2)7(11)8-4-6(9)10/h5H,3-4H2,1-2H3,(H,8,11)(H,9,10)

> <INCHI_KEY>
HOACIBQKYRHBOW-UHFFFAOYSA-N

> <FORMULA>
C7H13NO3

> <MOLECULAR_WEIGHT>
159.183

> <EXACT_MASS>
159.089543287

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
24

> <JCHEM_AVERAGE_POLARIZABILITY>
16.5527710618262

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
2

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
2-(2-methylbutanamido)acetic acid

> <ALOGPS_LOGP>
0.41

> <JCHEM_LOGP>
0.3594618363333333

> <ALOGPS_LOGS>
-1.25

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA>
15.85921724512119

> <JCHEM_PKA_STRONGEST_ACIDIC>
4.166376353767786

> <JCHEM_PKA_STRONGEST_BASIC>
-1.64156885582777

> <JCHEM_POLAR_SURFACE_AREA>
66.4

> <JCHEM_REFRACTIVITY>
39.2496

> <JCHEM_ROTATABLE_BOND_COUNT>
4

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
9.00e+00 g/l

> <JCHEM_TRADITIONAL_IUPAC>
2-methylbutyrylglycine

> <JCHEM_VEBER_RULE>
0

$$$$
Download

3D-SDF for NP0000232 (2-Methylbutyrylglycine)

HMDB0000339
     RDKit          3D
2-Methylbutyrylglycine
 24 23  0  0  0  0  0  0  0  0999 V2000
   -3.3634    0.4052    0.6312 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9082    0.2303    0.9676 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1226   -0.3679   -0.1729 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1714    0.4815   -1.4121 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3071   -0.4402    0.2637 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.6237   -0.0259    1.4237 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.3021   -0.9617   -0.5971 N   0  0  0  0  0  0  0  0  0  0  0  0
    2.6615   -1.0068   -0.1243 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.1210    0.3608    0.1950 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.3873    1.3660    0.0516 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.4404    0.4742    0.6738 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5215    1.3264    0.0678 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6955   -0.4815    0.0779 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.9120    0.4198    1.6168 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4281    1.1712    1.3061 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8269   -0.5053    1.8165 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4609   -1.4097   -0.3627 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0217    0.2254   -2.0761 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2296    0.2920   -1.9741 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2069    1.5451   -1.0984 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.0069   -1.2886   -1.5315 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.7165   -1.6222    0.7962 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.3523   -1.4776   -0.8508 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.9496    1.2897    0.3122 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  1  0
  3  5  1  0
  5  6  2  0
  5  7  1  0
  7  8  1  0
  8  9  1  0
  9 10  2  0
  9 11  1  0
  1 12  1  0
  1 13  1  0
  1 14  1  0
  2 15  1  0
  2 16  1  0
  3 17  1  0
  4 18  1  0
  4 19  1  0
  4 20  1  0
  7 21  1  0
  8 22  1  0
  8 23  1  0
 11 24  1  0
M  END
Download

PDB for NP0000232 (2-Methylbutyrylglycine)

HEADER    PROTEIN                                 26-MAY-19   NONE
TITLE     NULL                                                        
COMPND    MOLECULE: 2-Methylbutyrylglycine.mol                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   26-MAY-19         0                                  
HETATM    1  C   UNK     0       2.667   0.385   0.000  0.00  0.00           C+0
HETATM    2  N   UNK     0       0.000   0.385   0.000  0.00  0.00           N+0
HETATM    3  C   UNK     0       1.334  -0.385   0.000  0.00  0.00           C+0
HETATM    4  O   UNK     0       2.667   1.925   0.000  0.00  0.00           O+0
HETATM    5  O   UNK     0       4.001  -0.385   0.000  0.00  0.00           O+0
HETATM    6  C   UNK     0      -1.334  -0.385   0.000  0.00  0.00           C+0
HETATM    7  O   UNK     0      -1.334  -1.925   0.000  0.00  0.00           O+0
HETATM    8  C   UNK     0      -2.667   0.385   0.000  0.00  0.00           C+0
HETATM    9  C   UNK     0      -4.001  -0.385   0.000  0.00  0.00           C+0
HETATM   10  C   UNK     0      -4.001  -1.925   0.000  0.00  0.00           C+0
HETATM   11  C   UNK     0      -2.667   1.925   0.000  0.00  0.00           C+0
CONECT    1    3    4    5                                                  
CONECT    2    3    6                                                       
CONECT    3    2    1                                                       
CONECT    4    1                                                            
CONECT    5    1                                                            
CONECT    6    7    8    2                                                  
CONECT    7    6                                                            
CONECT    8    6    9   11                                                  
CONECT    9    8   10                                                       
CONECT   10    9                                                            
CONECT   11    8                                                            
MASTER        0    0    0    0    0    0    0    0   11    0   20    0
END
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3D PDB for NP0000232 (2-Methylbutyrylglycine)

COMPND    HMDB0000339
HETATM    1  C1  UNL     1      -3.363   0.405   0.631  1.00  0.00           C  
HETATM    2  C2  UNL     1      -1.908   0.230   0.968  1.00  0.00           C  
HETATM    3  C3  UNL     1      -1.123  -0.368  -0.173  1.00  0.00           C  
HETATM    4  C4  UNL     1      -1.171   0.481  -1.412  1.00  0.00           C  
HETATM    5  C5  UNL     1       0.307  -0.440   0.264  1.00  0.00           C  
HETATM    6  O1  UNL     1       0.624  -0.026   1.424  1.00  0.00           O  
HETATM    7  N1  UNL     1       1.302  -0.962  -0.597  1.00  0.00           N  
HETATM    8  C6  UNL     1       2.662  -1.007  -0.124  1.00  0.00           C  
HETATM    9  C7  UNL     1       3.121   0.361   0.195  1.00  0.00           C  
HETATM   10  O2  UNL     1       2.387   1.366   0.052  1.00  0.00           O  
HETATM   11  O3  UNL     1       4.440   0.474   0.674  1.00  0.00           O  
HETATM   12  H1  UNL     1      -3.521   1.326   0.068  1.00  0.00           H  
HETATM   13  H2  UNL     1      -3.696  -0.481   0.078  1.00  0.00           H  
HETATM   14  H3  UNL     1      -3.912   0.420   1.617  1.00  0.00           H  
HETATM   15  H4  UNL     1      -1.428   1.171   1.306  1.00  0.00           H  
HETATM   16  H5  UNL     1      -1.827  -0.505   1.816  1.00  0.00           H  
HETATM   17  H6  UNL     1      -1.461  -1.410  -0.363  1.00  0.00           H  
HETATM   18  H7  UNL     1      -2.022   0.225  -2.076  1.00  0.00           H  
HETATM   19  H8  UNL     1      -0.230   0.292  -1.974  1.00  0.00           H  
HETATM   20  H9  UNL     1      -1.207   1.545  -1.098  1.00  0.00           H  
HETATM   21  H10 UNL     1       1.007  -1.289  -1.531  1.00  0.00           H  
HETATM   22  H11 UNL     1       2.716  -1.622   0.796  1.00  0.00           H  
HETATM   23  H12 UNL     1       3.352  -1.478  -0.851  1.00  0.00           H  
HETATM   24  H13 UNL     1       4.950   1.290   0.312  1.00  0.00           H  
CONECT    1    2   12   13   14
CONECT    2    3   15   16
CONECT    3    4    5   17
CONECT    4   18   19   20
CONECT    5    6    6    7
CONECT    7    8   21
CONECT    8    9   22   23
CONECT    9   10   10   11
CONECT   11   24
END
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SMILES for NP0000232 (2-Methylbutyrylglycine)

CCC(C)C(=O)NCC(O)=O
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INCHI for NP0000232 (2-Methylbutyrylglycine)

InChI=1S/C7H13NO3/c1-3-5(2)7(11)8-4-6(9)10/h5H,3-4H2,1-2H3,(H,8,11)(H,9,10)
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View in JSmol
Synonyms
ValueSource
alpha-MethylbutyrylglycineChEBI
a-MethylbutyrylglycineGenerator
Α-methylbutyrylglycineGenerator
(2-Methyl-butyrylamino)-acetateHMDB
(2-Methyl-butyrylamino)-acetic acidHMDB
2-MBGHMDB
2-Methylbutyryl glycineHMDB
N-(2-Methylbutyryl)glycineHMDB
N-Sec-valerylglycineHMDB
2-MethylbutyrylglycineChEBI
Chemical FormulaC7H13NO3
Average Mass159.1830 Da
Monoisotopic Mass159.08954 Da
IUPAC Name2-(2-methylbutanamido)acetic acid
Traditional Name2-methylbutyrylglycine
CAS Registry Number52320-67-9
SMILES
CCC(C)C(=O)NCC(O)=O
InChI Identifier
InChI=1S/C7H13NO3/c1-3-5(2)7(11)8-4-6(9)10/h5H,3-4H2,1-2H3,(H,8,11)(H,9,10)
InChI KeyHOACIBQKYRHBOW-UHFFFAOYSA-N
Experimental Spectra
Spectrum TypeDescriptionDepositor EmailDepositor OrganizationDepositorDeposition DateView
1D NMR1H NMR Spectrum (1D, 500 MHz, CDCl3, experimental)Wishart LabWishart LabDavid Wishart2021-06-20View Spectrum
2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, CD3OD, experimental)Wishart LabWishart LabDavid Wishart2021-06-20View Spectrum
Predicted Spectra
Not Available
Chemical Shift Submissions
Not Available
Species
Species of Origin
Species NameSourceReference
Anas platyrhynchosFooDB
AnatidaeFooDB
Anser anserFooDB
Bison bisonFooDB
Bos taurusFooDB
Bos taurus X Bison bisonFooDB
Bubalus bubalisFooDB
Capra aegagrus hircusFooDB
CervidaeFooDB
Cervus canadensisFooDB
ColumbaFooDB
ColumbidaeFooDB
Dromaius novaehollandiaeFooDB
Equus caballusFooDB
Gallus gallusFooDB
Lagopus mutaFooDB
LeporidaeFooDB
Lepus timidusFooDB
Melanitta fuscaFooDB
Meleagris gallopavoFooDB
Numida meleagrisFooDB
OdocoileusFooDB
OryctolagusFooDB
Ovis ariesFooDB
PhasianidaeFooDB
Phasianus colchicusFooDB
Struthio camelusFooDB
Sus scrofaFooDB
Sus scrofa domesticaFooDB
Species Where Detected
Species NameSourceReference
Homo sapiens (Urine)KNApSAcK Database
Chemical Taxonomy
Description Belongs to the class of organic compounds known as n-acyl-alpha amino acids. N-acyl-alpha amino acids are compounds containing an alpha amino acid which bears an acyl group at its terminal nitrogen atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentN-acyl-alpha amino acids
Alternative Parents
Substituents
  • N-acyl-alpha-amino acid
  • Carboximidic acid
  • Carboximidic acid derivative
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Organic 1,3-dipolar compound
  • Propargyl-type 1,3-dipolar organic compound
  • Carbonyl group
  • Organooxygen compound
  • Organonitrogen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Organic nitrogen compound
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility9 g/LALOGPS
logP0.41ALOGPS
logP0.36ChemAxon
logS-1.2ALOGPS
pKa (Strongest Acidic)4.17ChemAxon
pKa (Strongest Basic)-1.6ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area66.4 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity39.25 m³·mol⁻¹ChemAxon
Polarizability16.55 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
HMDB IDHMDB0000339
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB021963
KNApSAcK IDC00052127
Chemspider ID168243
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID5328
PubChem Compound193872
PDB IDNot Available
ChEBI ID86366
Good Scents IDNot Available
References
General References
  1. Korman SH, Andresen BS, Zeharia A, Gutman A, Boneh A, Pitt JJ: 2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation. Clin Chem. 2005 Mar;51(3):610-7. Epub 2004 Dec 22. [PubMed:15615815 ]
  2. Tein I, Haslam RH, Rhead WJ, Bennett MJ, Becker LE, Vockley J: Short-chain acyl-CoA dehydrogenase deficiency: a cause of ophthalmoplegia and multicore myopathy. Neurology. 1999 Jan 15;52(2):366-72. [PubMed:9932958 ]
  3. Sweetman L, Weyler W, Nyhan WL, de Cespedes C, Loria AR, Estrada Y: Abnormal metabolites of isoleucine in a patient with propionyl-CoA carboxylase deficiency. Biomed Mass Spectrom. 1978 Mar;5(3):198-207. [PubMed:630060 ]
  4. Kanavin OJ, Woldseth B, Jellum E, Tvedt B, Andresen BS, Stromme P: 2-methylbutyryl-CoA dehydrogenase deficiency associated with autism and mental retardation: a case report. J Med Case Rep. 2007 Sep 20;1:98. [PubMed:17883863 ]
  5. Bouatra S, Aziat F, Mandal R, Guo AC, Wilson MR, Knox C, Bjorndahl TC, Krishnamurthy R, Saleem F, Liu P, Dame ZT, Poelzer J, Huynh J, Yallou FS, Psychogios N, Dong E, Bogumil R, Roehring C, Wishart DS: The human urine metabolome. PLoS One. 2013 Sep 4;8(9):e73076. doi: 10.1371/journal.pone.0073076. eCollection 2013. [PubMed:24023812 ]