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Showing NP-Card for Tiglylglycine (NP0001006)

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Record Information
Version2.0
Created at2005-11-16 15:48:42 UTC
Updated at2020-11-24 22:20:04 UTC
NP-MRD IDNP0001006
Secondary Accession NumbersNone
Natural Product Identification
Common NameTiglylglycine
DescriptionTiglylglycine 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. 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. Tiglylglycine is an intermediate product of the catabolism of isoleucine. An elevated level of tiglylglycine is identified in urine of patients with beta-ketothiolase deficiency or with disorders of propionate metabolism (PMID 7923765 ). Tiglyglycine is a biomarker for the consumption of cheese.
Structure
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MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for NP0001006 (Tiglylglycine)


  Mrv1652305271900312D          

 11 10  0  0  0  0            999 V2000
   14.3727   -6.9600    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   11.5150   -6.1350    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   12.2293   -4.8976    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   13.6583   -5.7224    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
   15.0871   -5.7224    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   14.3727   -6.1350    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   12.9438   -6.1350    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   15.0871   -4.8976    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   15.8017   -6.1350    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   16.5160   -5.7224    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   12.2293   -5.7224    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  6  2  0  0  0  0
  2 11  1  0  0  0  0
  3 11  2  0  0  0  0
  4  6  1  0  0  0  0
  4  7  1  0  0  0  0
  5  6  1  0  0  0  0
  5  8  1  0  0  0  0
  5  9  2  0  0  0  0
  7 11  1  0  0  0  0
  9 10  1  0  0  0  0
M  END
Download

3D MOL for NP0001006 (Tiglylglycine)

HMDB0000959
     RDKit          3D
Tiglylglycine
 22 21  0  0  0  0  0  0  0  0999 V2000
   -3.4406   -0.8509    0.4062 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9631   -1.0313    0.5243 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1080   -0.2222   -0.0744 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.6633    0.8940   -0.8843 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3406   -0.4170    0.0547 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7433   -1.3809    0.7548 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.2770    0.4289   -0.5715 N   0  0  0  0  0  0  0  0  0  0  0  0
    2.6877    0.2027   -0.4188 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.0243    0.2925    1.0214 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.1599    0.5305    1.8797 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.3468    0.1015    1.3911 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.9016   -1.4823    1.1942 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6844    0.2129    0.6151 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.7233   -1.1443   -0.6114 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5299   -1.8387    1.1099 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6168    0.5202   -1.3482 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9612    1.7398   -0.2136 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0137    1.2011   -1.7110 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.9456    1.2195   -1.1460 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.2839    0.9282   -1.0251 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.8945   -0.8294   -0.7666 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.9021    0.9253    1.5712 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  2  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
  4 16  1  0
  4 17  1  0
  4 18  1  0
  7 19  1  0
  8 20  1  0
  8 21  1  0
 11 22  1  0
M  END
Download

3D SDF for NP0001006 (Tiglylglycine)


  Mrv1652305271900312D          

 11 10  0  0  0  0            999 V2000
   14.3727   -6.9600    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   11.5150   -6.1350    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   12.2293   -4.8976    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   13.6583   -5.7224    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
   15.0871   -5.7224    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   14.3727   -6.1350    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   12.9438   -6.1350    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   15.0871   -4.8976    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   15.8017   -6.1350    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   16.5160   -5.7224    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   12.2293   -5.7224    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  6  2  0  0  0  0
  2 11  1  0  0  0  0
  3 11  2  0  0  0  0
  4  6  1  0  0  0  0
  4  7  1  0  0  0  0
  5  6  1  0  0  0  0
  5  8  1  0  0  0  0
  5  9  2  0  0  0  0
  7 11  1  0  0  0  0
  9 10  1  0  0  0  0
M  END
> <DATABASE_ID>
NP0001006

> <DATABASE_NAME>
NP-MRD

> <SMILES>
C\C=C(/C)C(=O)NCC(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C7H11NO3/c1-3-5(2)7(11)8-4-6(9)10/h3H,4H2,1-2H3,(H,8,11)(H,9,10)/b5-3+

> <INCHI_KEY>
WRUSVQOKJIDBLP-HWKANZROSA-N

> <FORMULA>
C7H11NO3

> <MOLECULAR_WEIGHT>
157.1671

> <EXACT_MASS>
157.073893223

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
22

> <JCHEM_AVERAGE_POLARIZABILITY>
15.97497515941016

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
2

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
2-[(2E)-2-methylbut-2-enamido]acetic acid

> <ALOGPS_LOGP>
0.33

> <JCHEM_LOGP>
0.2098495370000003

> <ALOGPS_LOGS>
-1.32

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA>
15.789016254103654

> <JCHEM_PKA_STRONGEST_ACIDIC>
4.09123447046491

> <JCHEM_PKA_STRONGEST_BASIC>
-0.48813886941777895

> <JCHEM_POLAR_SURFACE_AREA>
66.39999999999999

> <JCHEM_REFRACTIVITY>
40.1255

> <JCHEM_ROTATABLE_BOND_COUNT>
3

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
7.52e+00 g/l

> <JCHEM_TRADITIONAL_IUPAC>
[(2E)-2-methylbut-2-enamido]acetic acid

> <JCHEM_VEBER_RULE>
0

$$$$
Download

3D-SDF for NP0001006 (Tiglylglycine)

HMDB0000959
     RDKit          3D
Tiglylglycine
 22 21  0  0  0  0  0  0  0  0999 V2000
   -3.4406   -0.8509    0.4062 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9631   -1.0313    0.5243 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1080   -0.2222   -0.0744 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.6633    0.8940   -0.8843 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3406   -0.4170    0.0547 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7433   -1.3809    0.7548 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.2770    0.4289   -0.5715 N   0  0  0  0  0  0  0  0  0  0  0  0
    2.6877    0.2027   -0.4188 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.0243    0.2925    1.0214 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.1599    0.5305    1.8797 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.3468    0.1015    1.3911 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.9016   -1.4823    1.1942 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6844    0.2129    0.6151 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.7233   -1.1443   -0.6114 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5299   -1.8387    1.1099 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6168    0.5202   -1.3482 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9612    1.7398   -0.2136 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0137    1.2011   -1.7110 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.9456    1.2195   -1.1460 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.2839    0.9282   -1.0251 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.8945   -0.8294   -0.7666 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.9021    0.9253    1.5712 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  2  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
  4 16  1  0
  4 17  1  0
  4 18  1  0
  7 19  1  0
  8 20  1  0
  8 21  1  0
 11 22  1  0
M  END
Download

PDB for NP0001006 (Tiglylglycine)

HEADER    PROTEIN                                 27-MAY-19   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   27-MAY-19         0                                  
HETATM    1  O   UNK     0      26.829 -12.992   0.000  0.00  0.00           O+0
HETATM    2  O   UNK     0      21.495 -11.452   0.000  0.00  0.00           O+0
HETATM    3  O   UNK     0      22.828  -9.142   0.000  0.00  0.00           O+0
HETATM    4  N   UNK     0      25.496 -10.682   0.000  0.00  0.00           N+0
HETATM    5  C   UNK     0      28.163 -10.682   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0      26.829 -11.452   0.000  0.00  0.00           C+0
HETATM    7  C   UNK     0      24.162 -11.452   0.000  0.00  0.00           C+0
HETATM    8  C   UNK     0      28.163  -9.142   0.000  0.00  0.00           C+0
HETATM    9  C   UNK     0      29.496 -11.452   0.000  0.00  0.00           C+0
HETATM   10  C   UNK     0      30.830 -10.682   0.000  0.00  0.00           C+0
HETATM   11  C   UNK     0      22.828 -10.682   0.000  0.00  0.00           C+0
CONECT    1    6                                                            
CONECT    2   11                                                            
CONECT    3   11                                                            
CONECT    4    6    7                                                       
CONECT    5    6    8    9                                                  
CONECT    6    1    4    5                                                  
CONECT    7    4   11                                                       
CONECT    8    5                                                            
CONECT    9    5   10                                                       
CONECT   10    9                                                            
CONECT   11    2    3    7                                                  
MASTER        0    0    0    0    0    0    0    0   11    0   20    0
END
Download

3D PDB for NP0001006 (Tiglylglycine)

COMPND    HMDB0000959
HETATM    1  C1  UNL     1      -3.441  -0.851   0.406  1.00  0.00           C  
HETATM    2  C2  UNL     1      -1.963  -1.031   0.524  1.00  0.00           C  
HETATM    3  C3  UNL     1      -1.108  -0.222  -0.074  1.00  0.00           C  
HETATM    4  C4  UNL     1      -1.663   0.894  -0.884  1.00  0.00           C  
HETATM    5  C5  UNL     1       0.341  -0.417   0.055  1.00  0.00           C  
HETATM    6  O1  UNL     1       0.743  -1.381   0.755  1.00  0.00           O  
HETATM    7  N1  UNL     1       1.277   0.429  -0.572  1.00  0.00           N  
HETATM    8  C6  UNL     1       2.688   0.203  -0.419  1.00  0.00           C  
HETATM    9  C7  UNL     1       3.024   0.293   1.021  1.00  0.00           C  
HETATM   10  O2  UNL     1       2.160   0.530   1.880  1.00  0.00           O  
HETATM   11  O3  UNL     1       4.347   0.101   1.391  1.00  0.00           O  
HETATM   12  H1  UNL     1      -3.902  -1.482   1.194  1.00  0.00           H  
HETATM   13  H2  UNL     1      -3.684   0.213   0.615  1.00  0.00           H  
HETATM   14  H3  UNL     1      -3.723  -1.144  -0.611  1.00  0.00           H  
HETATM   15  H4  UNL     1      -1.530  -1.839   1.110  1.00  0.00           H  
HETATM   16  H5  UNL     1      -2.617   0.520  -1.348  1.00  0.00           H  
HETATM   17  H6  UNL     1      -1.961   1.740  -0.214  1.00  0.00           H  
HETATM   18  H7  UNL     1      -1.014   1.201  -1.711  1.00  0.00           H  
HETATM   19  H8  UNL     1       0.946   1.219  -1.146  1.00  0.00           H  
HETATM   20  H9  UNL     1       3.284   0.928  -1.025  1.00  0.00           H  
HETATM   21  H10 UNL     1       2.894  -0.829  -0.767  1.00  0.00           H  
HETATM   22  H11 UNL     1       4.902   0.925   1.571  1.00  0.00           H  
CONECT    1    2   12   13   14
CONECT    2    3    3   15
CONECT    3    4    5
CONECT    4   16   17   18
CONECT    5    6    6    7
CONECT    7    8   19
CONECT    8    9   20   21
CONECT    9   10   10   11
CONECT   11   22
END
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SMILES for NP0001006 (Tiglylglycine)

C\C=C(/C)C(=O)NCC(O)=O
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INCHI for NP0001006 (Tiglylglycine)

InChI=1S/C7H11NO3/c1-3-5(2)7(11)8-4-6(9)10/h3H,4H2,1-2H3,(H,8,11)(H,9,10)/b5-3+
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View in JSmol
Synonyms
ValueSource
N-TiglylglycineChEBI
(e)-N-(2-Methyl-1-oxo-2-butenyl)-glycineHMDB
N-((e)-2-Methyl-but-2-enoyl)-glycineHMDB
N-TigloylglycineHMDB
Chemical FormulaC7H11NO3
Average Mass157.1671 Da
Monoisotopic Mass157.07389 Da
IUPAC Name2-[(2E)-2-methylbut-2-enamido]acetic acid
Traditional Name[(2E)-2-methylbut-2-enamido]acetic acid
CAS Registry Number35842-45-6
SMILES
C\C=C(/C)C(=O)NCC(O)=O
InChI Identifier
InChI=1S/C7H11NO3/c1-3-5(2)7(11)8-4-6(9)10/h3H,4H2,1-2H3,(H,8,11)(H,9,10)/b5-3+
InChI KeyWRUSVQOKJIDBLP-HWKANZROSA-N
Experimental Spectra
Spectrum TypeDescriptionDepositor EmailDepositor OrganizationDepositorDeposition DateView
1D NMR1H NMR Spectrum (1D, 500 MHz, H2O, experimental)Wishart LabWishart LabDavid Wishart2021-06-20View Spectrum
2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, 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
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
  • N-acyl-amine
  • Secondary carboxylic acid amide
  • Carboxamide group
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Organic nitrogen compound
  • Organic oxygen compound
  • Organopnictogen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • 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 Solubility7.52 g/LALOGPS
logP0.33ALOGPS
logP0.21ChemAxon
logS-1.3ALOGPS
pKa (Strongest Acidic)4.09ChemAxon
pKa (Strongest Basic)-0.49ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area66.4 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity40.13 m³·mol⁻¹ChemAxon
Polarizability15.97 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
HMDB IDHMDB0000959
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB022337
KNApSAcK IDNot Available
Chemspider ID4945715
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID4246
PubChem Compound6441567
PDB IDNot Available
ChEBI ID73018
Good Scents IDNot Available
References
General References
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  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. Hagen T, Korson MS, Sakamoto M, Evans JE: A GC/MS/MS screening method for multiple organic acidemias from urine specimens. Clin Chim Acta. 1999 May;283(1-2):77-88. [PubMed:10404733 ]
  4. Poll-The BT, Wanders RJ, Ruiter JP, Ofman R, Majoie CB, Barth PG, Duran M: Spastic diplegia and periventricular white matter abnormalities in 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency, a defect of isoleucine metabolism: differential diagnosis with hypoxic-ischemic brain diseases. Mol Genet Metab. 2004 Apr;81(4):295-9. [PubMed:15059617 ]
  5. 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 ]
  6. Zschocke J, Ruiter JP, Brand J, Lindner M, Hoffmann GF, Wanders RJ, Mayatepek E: Progressive infantile neurodegeneration caused by 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency: a novel inborn error of branched-chain fatty acid and isoleucine metabolism. Pediatr Res. 2000 Dec;48(6):852-5. [PubMed:11102558 ]
  7. Bennett MJ, Powell S, Swartling DJ, Gibson KM: Tiglylglycine excreted in urine in disorders of isoleucine metabolism and the respiratory chain measured by stable isotope dilution GC-MS. Clin Chem. 1994 Oct;40(10):1879-83. [PubMed:7923765 ]
  8. 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 ]