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Showing NP-Card for 3-Hydroxymethylglutaric acid (NP0000411)

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Record Information
Version1.0
Created at2005-11-16 15:48:42 UTC
Updated at2020-11-24 22:15:17 UTC
NP-MRD IDNP0000411
Secondary Accession NumbersNone
Natural Product Identification
Common Name3-Hydroxymethylglutaric acid
Description3-Hydroxymethylglutaric acid is an "off-product" intermediate in the leucine degradation process. It is produced by defective or inefficient versions of 3-hydroxy-3-methylglutaryl-CoA lyase, an enzyme that normally catalyzes the conversion of 3-hydroxy-3-methylglutaryl-CoA to acetyl-CoA and acetoacetate. If this enzyme is defective, 3-hydroxy-3-methylglutaryl-CoA will accumulate in the mitochondria. Increased concentrations of 3-hydroxy-3-methylglutaryl-CoA can lead to a disruption of the esterified CoA:Free CoA ratio and ultimately to mitochondrial toxicity. Detoxification of these CoA end products occurs via the transfer of the 3-hydroxymethylglutaryl moiety to carnitine, forming 3-hydroxymethylglutaric-carnitine, which is then transferred across the inner mitochondrial membrane where 3-hydroxymethylglutaric acid is released as the free acid. 3-Hydroxymethylglutaric acid has been found to accumulate in the urine of patients affected by 3-Hydroxy-3-methylglutaric aciduria, a rare inborn error of metabolism (OMIM: 246450 ). 3-Hydroxy-3-methylglutaric aciduria is caused by significantly reduced enzyme activity of the intramitochondrial 3-hydroxy-3-methylglutaryl-CoA lyase (EC 4.1.3.4), The enzyme that catalyzes the final step of leucine degradation. This enzyme also plays a key role in ketone body formation. The profile of urinary organic acids for individuals with 3-hydroxy-3-methylglutaric aciduria is different from that of the other identified defects of leucine degradation, such as maple syrup urine disease (OMIM: 248600 ), Isovaleric acidemia (OMIM: 243500 ), And methylcrotonylglycinemia (OMIM: 210200 ). The urinary organic acid profile of 3-hydroxy-3-methylglutaric aciduria includes elevated concentrations of 3-hydroxy-3-isovaleric, 3-hydroxy-3-methylglutaric, 3-methylglutaconic, and 3-methylglutaric acids (PMID: 10916782 , 9658458 , 3063529 ). Clinical manifestations of 3-hydroxy-3-methylglutaric aciduria include hepatomegaly, lethargy, coma, and apnea. Biochemically, there is a characteristic absence of ketosis with hypoglycemia, acidosis, hypertransaminasemia, and variable hyperammonemia. Therefore, when present in sufficiently high concentrations, 3-hydroxymethylglutaric acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. As noted above, chronically high levels of 3-hydroxymethylglutaric acid are associated with the inborn error of metabolism 3-hydroxy-3-methylglutaryl-CoA lyase deficiency. 3-Hydroxymethylglutaric acid is an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures.
Structure
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MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for NP0000411 (3-Hydroxymethylglutaric acid)


  Mrv1652305261923572D          

 11 10  0  0  0  0            999 V2000
 9999.2905 9997.9678    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9998.5756 9998.3795    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9997.8608 9997.9678    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9997.1459 9998.3795    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9996.4310 9997.9678    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9997.1459 9999.2054    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.9892 9999.0960    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9998.1638 9999.0960    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10000.0084 9998.3795    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10000.7223 9997.9678    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10000.0084 9999.2054    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  9  1  0  0  0  0
  2  3  1  0  0  0  0
  2  7  1  0  0  0  0
  2  8  1  0  0  0  0
  3  4  1  0  0  0  0
  4  5  1  0  0  0  0
  4  6  2  0  0  0  0
  9 10  1  0  0  0  0
  9 11  2  0  0  0  0
M  END
Download

3D MOL for NP0000411 (3-Hydroxymethylglutaric acid)

HMDB0000355
     RDKit          3D
3-Hydroxymethylglutaric acid
 21 20  0  0  0  0  0  0  0  0999 V2000
   -0.2537    1.0361   -1.5362 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.0018   -0.1340   -0.6708 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.1538   -1.1921   -1.7139 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.3140   -0.2263    0.0025 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.7498    0.8221    0.8872 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.1617    1.8929    1.0942 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.9446    0.6400    1.5902 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1052   -0.6915    0.1211 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8242    0.1036    1.0798 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7311    1.3111    1.3537 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8015   -0.5708    1.8495 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5155    1.9691   -1.0058 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.6801    1.2509   -2.1043 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0179    0.7906   -2.3122 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6842   -1.1227   -2.2457 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3737   -1.1993    0.5951 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.1661   -0.3866   -0.7434 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.6328   -0.0496    1.3287 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9155   -1.1549   -0.5437 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7466   -1.6272    0.6706 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5830   -1.4614    2.3031 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  2  0
  5  7  1  0
  2  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
  3 15  1  0
  4 16  1  0
  4 17  1  0
  7 18  1  0
  8 19  1  0
  8 20  1  0
 11 21  1  0
M  END
Download

3D SDF for NP0000411 (3-Hydroxymethylglutaric acid)


  Mrv1652305261923572D          

 11 10  0  0  0  0            999 V2000
 9999.2905 9997.9678    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9998.5756 9998.3795    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9997.8608 9997.9678    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9997.1459 9998.3795    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9996.4310 9997.9678    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9997.1459 9999.2054    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.9892 9999.0960    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9998.1638 9999.0960    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10000.0084 9998.3795    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10000.7223 9997.9678    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10000.0084 9999.2054    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  9  1  0  0  0  0
  2  3  1  0  0  0  0
  2  7  1  0  0  0  0
  2  8  1  0  0  0  0
  3  4  1  0  0  0  0
  4  5  1  0  0  0  0
  4  6  2  0  0  0  0
  9 10  1  0  0  0  0
  9 11  2  0  0  0  0
M  END
> <DATABASE_ID>
NP0000411

> <DATABASE_NAME>
NP-MRD

> <SMILES>
CC(O)(CC(O)=O)CC(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C6H10O5/c1-6(11,2-4(7)8)3-5(9)10/h11H,2-3H2,1H3,(H,7,8)(H,9,10)

> <INCHI_KEY>
NPOAOTPXWNWTSH-UHFFFAOYSA-N

> <FORMULA>
C6H10O5

> <MOLECULAR_WEIGHT>
162.1406

> <EXACT_MASS>
162.05282343

> <JCHEM_ACCEPTOR_COUNT>
5

> <JCHEM_ATOM_COUNT>
21

> <JCHEM_AVERAGE_POLARIZABILITY>
14.545141112043254

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
3

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
3-hydroxy-3-methylpentanedioic acid

> <ALOGPS_LOGP>
-0.88

> <JCHEM_LOGP>
-0.7482412409999999

> <ALOGPS_LOGS>
0.23

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-2

> <JCHEM_PKA>
4.435120601836896

> <JCHEM_PKA_STRONGEST_ACIDIC>
3.6777896096277964

> <JCHEM_PKA_STRONGEST_BASIC>
-3.0082279564358467

> <JCHEM_POLAR_SURFACE_AREA>
94.83000000000001

> <JCHEM_REFRACTIVITY>
34.1377

> <JCHEM_ROTATABLE_BOND_COUNT>
4

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
2.75e+02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
meglutol

> <JCHEM_VEBER_RULE>
0

$$$$
Download

3D-SDF for NP0000411 (3-Hydroxymethylglutaric acid)

HMDB0000355
     RDKit          3D
3-Hydroxymethylglutaric acid
 21 20  0  0  0  0  0  0  0  0999 V2000
   -0.2537    1.0361   -1.5362 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.0018   -0.1340   -0.6708 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.1538   -1.1921   -1.7139 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.3140   -0.2263    0.0025 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.7498    0.8221    0.8872 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.1617    1.8929    1.0942 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.9446    0.6400    1.5902 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1052   -0.6915    0.1211 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8242    0.1036    1.0798 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7311    1.3111    1.3537 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8015   -0.5708    1.8495 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5155    1.9691   -1.0058 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.6801    1.2509   -2.1043 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0179    0.7906   -2.3122 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6842   -1.1227   -2.2457 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3737   -1.1993    0.5951 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.1661   -0.3866   -0.7434 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.6328   -0.0496    1.3287 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9155   -1.1549   -0.5437 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7466   -1.6272    0.6706 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5830   -1.4614    2.3031 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  2  0
  5  7  1  0
  2  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
  3 15  1  0
  4 16  1  0
  4 17  1  0
  7 18  1  0
  8 19  1  0
  8 20  1  0
 11 21  1  0
M  END
Download

PDB for NP0000411 (3-Hydroxymethylglutaric acid)

HEADER    PROTEIN                                 26-MAY-19   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   26-MAY-19         0                                  
HETATM    1  C   UNK     0    8665.3428662.873   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0    8664.0088663.642   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0    8662.6748662.873   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0    8661.3398663.642   0.000  0.00  0.00           C+0
HETATM    5  O   UNK     0    8660.0058662.873   0.000  0.00  0.00           O+0
HETATM    6  O   UNK     0    8661.3398665.183   0.000  0.00  0.00           O+0
HETATM    7  C   UNK     0    8664.7808664.979   0.000  0.00  0.00           C+0
HETATM    8  O   UNK     0    8663.2398664.979   0.000  0.00  0.00           O+0
HETATM    9  C   UNK     0    8666.6828663.642   0.000  0.00  0.00           C+0
HETATM   10  O   UNK     0    8668.0158662.873   0.000  0.00  0.00           O+0
HETATM   11  O   UNK     0    8666.6828665.183   0.000  0.00  0.00           O+0
CONECT    1    2    9                                                       
CONECT    2    1    3    7    8                                             
CONECT    3    2    4                                                       
CONECT    4    3    5    6                                                  
CONECT    5    4                                                            
CONECT    6    4                                                            
CONECT    7    2                                                            
CONECT    8    2                                                            
CONECT    9    1   10   11                                                  
CONECT   10    9                                                            
CONECT   11    9                                                            
MASTER        0    0    0    0    0    0    0    0   11    0   20    0
END
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3D PDB for NP0000411 (3-Hydroxymethylglutaric acid)

COMPND    HMDB0000355
HETATM    1  C1  UNL     1      -0.254   1.036  -1.536  1.00  0.00           C  
HETATM    2  C2  UNL     1       0.002  -0.134  -0.671  1.00  0.00           C  
HETATM    3  O1  UNL     1       0.154  -1.192  -1.714  1.00  0.00           O  
HETATM    4  C3  UNL     1       1.314  -0.226   0.003  1.00  0.00           C  
HETATM    5  C4  UNL     1       1.750   0.822   0.887  1.00  0.00           C  
HETATM    6  O2  UNL     1       1.162   1.893   1.094  1.00  0.00           O  
HETATM    7  O3  UNL     1       2.945   0.640   1.590  1.00  0.00           O  
HETATM    8  C5  UNL     1      -1.105  -0.691   0.121  1.00  0.00           C  
HETATM    9  C6  UNL     1      -1.824   0.104   1.080  1.00  0.00           C  
HETATM   10  O4  UNL     1      -1.731   1.311   1.354  1.00  0.00           O  
HETATM   11  O5  UNL     1      -2.801  -0.571   1.849  1.00  0.00           O  
HETATM   12  H1  UNL     1      -0.515   1.969  -1.006  1.00  0.00           H  
HETATM   13  H2  UNL     1       0.680   1.251  -2.104  1.00  0.00           H  
HETATM   14  H3  UNL     1      -1.018   0.791  -2.312  1.00  0.00           H  
HETATM   15  H4  UNL     1      -0.684  -1.123  -2.246  1.00  0.00           H  
HETATM   16  H5  UNL     1       1.374  -1.199   0.595  1.00  0.00           H  
HETATM   17  H6  UNL     1       2.166  -0.387  -0.743  1.00  0.00           H  
HETATM   18  H7  UNL     1       3.633  -0.050   1.329  1.00  0.00           H  
HETATM   19  H8  UNL     1      -1.915  -1.155  -0.544  1.00  0.00           H  
HETATM   20  H9  UNL     1      -0.747  -1.627   0.671  1.00  0.00           H  
HETATM   21  H10 UNL     1      -2.583  -1.461   2.303  1.00  0.00           H  
CONECT    1    2   12   13   14
CONECT    2    3    4    8
CONECT    3   15
CONECT    4    5   16   17
CONECT    5    6    6    7
CONECT    7   18
CONECT    8    9   19   20
CONECT    9   10   10   11
CONECT   11   21
END
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SMILES for NP0000411 (3-Hydroxymethylglutaric acid)

CC(O)(CC(O)=O)CC(O)=O
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INCHI for NP0000411 (3-Hydroxymethylglutaric acid)

InChI=1S/C6H10O5/c1-6(11,2-4(7)8)3-5(9)10/h11H,2-3H2,1H3,(H,7,8)(H,9,10)
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View in JSmol
Synonyms
ValueSource
(S)-3-Hydroxy-3-methylglutaric acidChEBI
(S)-MeglutolChEBI
3-HYDROXY-3-methyl-glutarIC ACIDChEBI
3-Hydroxy-3-methylpentanedioic acidChEBI
beta-Hydroxy-beta-methylglutaric acidChEBI
Dicrotalic acidChEBI
MeglutolChEBI
MeglutolumChEBI
3-Hydorxy-3-methylglutaric acidKegg
(S)-3-Hydroxy-3-methylglutarateGenerator
3-HYDROXY-3-methyl-glutarateGenerator
3-Hydroxy-3-methylpentanedioateGenerator
b-Hydroxy-b-methylglutarateGenerator
b-Hydroxy-b-methylglutaric acidGenerator
beta-Hydroxy-beta-methylglutarateGenerator
Β-hydroxy-β-methylglutarateGenerator
Β-hydroxy-β-methylglutaric acidGenerator
DicrotalateGenerator
3-Hydorxy-3-methylglutarateGenerator
3-HydroxymethylglutarateGenerator
3-Hydroxy-3-methylglutarateHMDB
3-Hydroxy-3-methylglutaric acidHMDB
3-Methyl-3-hydroxyglutarateHMDB
3-Methyl-3-hydroxyglutaric acidHMDB
CB 337HMDB
3 Hydroxy 3 methylpentanedioic acidHMDB
Acid, 3-hydroxy-3-methylglutaricHMDB
Acid, 3-hydroxy-3-methylpentanedioicHMDB
beta Hydroxy beta methylglutarateHMDB
3 Hydroxy 3 methylglutaric acidHMDB
3-Methyl-3-hydroxypentanedioateHMDB
3-Methyl-3-hydroxypentanedioic acidHMDB
HMGHMDB
HMGAHMDB
LipoglutarenHMDB
MedroglutarateHMDB
Medroglutaric acidHMDB
Chemical FormulaC6H10O5
Average Mass162.1406 Da
Monoisotopic Mass162.05282 Da
IUPAC Name3-hydroxy-3-methylpentanedioic acid
Traditional Namemeglutol
CAS Registry Number503-49-1
SMILES
CC(O)(CC(O)=O)CC(O)=O
InChI Identifier
InChI=1S/C6H10O5/c1-6(11,2-4(7)8)3-5(9)10/h11H,2-3H2,1H3,(H,7,8)(H,9,10)
InChI KeyNPOAOTPXWNWTSH-UHFFFAOYSA-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
Arabidopsis thalianaLOTUS Database
Avena sativa L.FooDB
Bison bisonFooDB
Bos taurusFooDB
Bos taurus X Bison bisonFooDB
Bubalus bubalisFooDB
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Chemical Taxonomy
Description Belongs to the class of organic compounds known as hydroxy fatty acids. These are fatty acids in which the chain bears a hydroxyl group.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acids and conjugates
Direct ParentHydroxy fatty acids
Alternative Parents
Substituents
  • Methyl-branched fatty acid
  • Short-chain hydroxy acid
  • Hydroxy fatty acid
  • Branched fatty acid
  • Dicarboxylic acid or derivatives
  • Tertiary alcohol
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point105 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility275 g/LALOGPS
logP-0.88ALOGPS
logP-0.75ChemAxon
logS0.23ALOGPS
pKa (Strongest Acidic)3.68ChemAxon
pKa (Strongest Basic)-3ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area94.83 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity34.14 m³·mol⁻¹ChemAxon
Polarizability14.55 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
HMDB IDHMDB0000355
DrugBank IDDB04377
Phenol Explorer Compound IDNot Available
FoodDB IDFDB004207
KNApSAcK IDC00001187
Chemspider ID1600
KEGG Compound IDC03761
BioCyc IDCPD-547
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID5344
PubChem Compound1662
PDB IDNot Available
ChEBI ID16831
Good Scents IDNot Available
References
General References
  1. Fu Z, Runquist JA, Forouhar F, Hussain M, Hunt JF, Miziorko HM, Kim JJ: Crystal structure of human 3-hydroxy-3-methylglutaryl-CoA Lyase: insights into catalysis and the molecular basis for hydroxymethylglutaric aciduria. J Biol Chem. 2006 Mar 17;281(11):7526-32. Epub 2005 Dec 5. [PubMed:16330550 ]
  2. Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed:8087979 ]
  3. Mitchell GA, Jakobs C, Gibson KM, Robert MF, Burlina A, Dionisi-Vici C, Dallaire L: Molecular prenatal diagnosis of 3-hydroxy-3-methylglutaryl CoA lyase deficiency. Prenat Diagn. 1995 Aug;15(8):725-9. [PubMed:7479590 ]
  4. Wysocki SJ, Wilkinson SP, Hahnel R, Wong CY, Panegyres PK: 3-Hydroxy-3-methylglutaric aciduria, combined with 3-methylglutaconic aciduria. Clin Chim Acta. 1976 Aug 2;70(3):399-406. [PubMed:947633 ]
  5. Tuchman M, McCann MT, Johnson PE, Lemieux B: Screening newborns for multiple organic acidurias in dried filter paper urine samples: method development. Pediatr Res. 1991 Oct;30(4):315-21. [PubMed:1956713 ]
  6. Truscott RJ, Halpern B, Wysocki SJ, Hahnel R, Wilcken B: Studies on a child suspected of having a dficiency in 3-hydroxy-3-methylglutaryl-Co A lyase. Clin Chim Acta. 1979 Jul 2;95(1):11-16. [PubMed:509721 ]
  7. Koling S, Kalhoff H, Schauerte P, Lehnert W, Diekmann L: [3-hydroxy-3-methylglutaraciduria (case report of a female Turkish sisters with 3-hydroxy-3- methylglutaryl-Coenzyme A lyase deficiency]. Klin Padiatr. 2000 May-Jun;212(3):113-6. [PubMed:10916782 ]
  8. Eiris J, Ribes A, Fernandez-Prieto R, Rodriguez-Garcia J, Rodriguez-Segade S, Castro-Gago M: [3-hydroxy-3-methylglutaric aciduria and recurrent Reye-like syndrome]. Rev Neurol. 1998 Jun;26(154):911-4. [PubMed:9658458 ]
  9. Gibson KM, Breuer J, Nyhan WL: 3-Hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: review of 18 reported patients. Eur J Pediatr. 1988 Dec;148(3):180-6. [PubMed:3063529 ]