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Showing metabocard for Oxoadipic acid (HMDB0000225)

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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-05-30 20:55:55 UTC
HMDB IDHMDB0000225
Secondary Accession Numbers
  • HMDB00225
Metabolite Identification
Common NameOxoadipic acid
Description2-Oxoadipic acid is produced from lysine in the cytosol of cells via the saccharopine and the pipecolic acid pathways. Catabolites of hydroxylysine and tryptophan enter these pathways as 2-aminoadipic- -semialdehyde and 2-oxoadipate, respectively. In the matrix of mitochondria, 2-oxoadipate is decarboxylated to glutaryl-CoA by the 2-oxoadipate dehydrogenase complex and then converted to acetyl-CoA. 2-Oxoadipic aciduria is an in-born error of metabolism of lysine, tryptophan, and hydroxylysine, in which abnormal quantities of 2-aminoadipic acid are found in body fluids along with 2-oxoadipic acid. Patients with 2-Oxoadipic acidemias are mentally retarded with hypotonia or seizures. 2-Oxoadipic aciduria can occur in patients with Kearns-Sayre Syndrome, a progressive disorder with onset prior to 20 years of age in which multiple organ systems are affected, including progressive external ophthalmoplegia, retinopathy, and the age of onset, and these are associated classically with abnormalities in cardiac conduction, cerebellar signs, and elevated cerebrospinal fluid protein (PMID: 10655159 , 16183823 , 11083877 ). Oxoadipic acid is found to be associated with alpha-aminoadipic aciduria, which is an inborn error of metabolism.
Structure
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MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0000225 (Oxoadipic acid)

Oxoadipic acid.mol
  Mrv1652309032023582D          

 11 10  0  0  0  0            999 V2000
   -1.7862    0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0717   -0.2062    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3572    0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3572   -0.2062    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.0717    0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.7862   -0.2062    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7862    1.0313    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5006   -0.2062    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.7862   -1.0312    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.5006    0.2063    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0717   -1.0313    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  2  3  1  0  0  0  0
  3  4  1  0  0  0  0
  4  5  1  0  0  0  0
  5  6  1  0  0  0  0
  1  7  2  0  0  0  0
  1  8  1  0  0  0  0
  6  9  2  0  0  0  0
  6 10  1  0  0  0  0
  2 11  2  0  0  0  0
M  END
Download

3D MOL for HMDB0000225 (Oxoadipic acid)

HMDB0000225
     RDKit          3D
Oxoadipic acid
 19 18  0  0  0  0  0  0  0  0999 V2000
    1.6355   -1.5653    0.7580 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.3373   -0.7801    0.1040 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.7175   -0.7887    0.3479 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.8379    0.1622   -0.9098 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3498    0.0980   -0.9932 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3601    0.4780    0.2611 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8288    0.3805    0.0656 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2566    0.0161   -1.0517 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7888    0.6891    1.1130 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3683    1.0588    2.2509 O   0  0  0  0  0  0  0  0  0  0  0  0
   -4.1586    0.5976    0.9255 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.0285   -0.5239    1.2730 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.2141    1.1828   -0.6551 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.2714   -0.0604   -1.9110 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.0062    0.7644   -1.8307 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.0935   -0.9555   -1.2825 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1713    1.5833    0.4175 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0496   -0.1067    1.1278 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.5096    0.4050   -0.0103 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  2  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  1  0
  6  7  1  0
  7  8  2  0
  7  9  1  0
  9 10  2  0
  9 11  1  0
  3 12  1  0
  4 13  1  0
  4 14  1  0
  5 15  1  0
  5 16  1  0
  6 17  1  0
  6 18  1  0
 11 19  1  0
M  END
Download

3D SDF for HMDB0000225 (Oxoadipic acid)

Oxoadipic acid.mol
  Mrv1652309032023582D          

 11 10  0  0  0  0            999 V2000
   -1.7862    0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0717   -0.2062    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3572    0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3572   -0.2062    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.0717    0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.7862   -0.2062    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7862    1.0313    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5006   -0.2062    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.7862   -1.0312    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.5006    0.2063    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0717   -1.0313    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  2  3  1  0  0  0  0
  3  4  1  0  0  0  0
  4  5  1  0  0  0  0
  5  6  1  0  0  0  0
  1  7  2  0  0  0  0
  1  8  1  0  0  0  0
  6  9  2  0  0  0  0
  6 10  1  0  0  0  0
  2 11  2  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0000225

> <DATABASE_NAME>
hmdb

> <SMILES>
OC(=O)CCCC(=O)C(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C6H8O5/c7-4(6(10)11)2-1-3-5(8)9/h1-3H2,(H,8,9)(H,10,11)

> <INCHI_KEY>
FGSBNBBHOZHUBO-UHFFFAOYSA-N

> <FORMULA>
C6H8O5

> <MOLECULAR_WEIGHT>
160.1247

> <EXACT_MASS>
160.037173366

> <JCHEM_ACCEPTOR_COUNT>
5

> <JCHEM_ATOM_COUNT>
19

> <JCHEM_AVERAGE_POLARIZABILITY>
14.159721281418744

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
2

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
2-oxohexanedioic acid

> <ALOGPS_LOGP>
-0.37

> <JCHEM_LOGP>
0.33530536533333316

> <ALOGPS_LOGS>
-0.83

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-2

> <JCHEM_PKA>
4.035296902862189

> <JCHEM_PKA_STRONGEST_ACIDIC>
2.842680623658864

> <JCHEM_PKA_STRONGEST_BASIC>
-9.657072598097674

> <JCHEM_POLAR_SURFACE_AREA>
91.66999999999999

> <JCHEM_REFRACTIVITY>
33.484

> <JCHEM_ROTATABLE_BOND_COUNT>
5

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
2.34e+01 g/l

> <JCHEM_TRADITIONAL_IUPAC>
oxoadipate

> <JCHEM_VEBER_RULE>
0

$$$$
Download

3D-SDF for HMDB0000225 (Oxoadipic acid)

HMDB0000225
     RDKit          3D
Oxoadipic acid
 19 18  0  0  0  0  0  0  0  0999 V2000
    1.6355   -1.5653    0.7580 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.3373   -0.7801    0.1040 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.7175   -0.7887    0.3479 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.8379    0.1622   -0.9098 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3498    0.0980   -0.9932 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3601    0.4780    0.2611 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8288    0.3805    0.0656 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2566    0.0161   -1.0517 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7888    0.6891    1.1130 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3683    1.0588    2.2509 O   0  0  0  0  0  0  0  0  0  0  0  0
   -4.1586    0.5976    0.9255 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.0285   -0.5239    1.2730 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.2141    1.1828   -0.6551 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.2714   -0.0604   -1.9110 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.0062    0.7644   -1.8307 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.0935   -0.9555   -1.2825 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1713    1.5833    0.4175 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0496   -0.1067    1.1278 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.5096    0.4050   -0.0103 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  2  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  1  0
  6  7  1  0
  7  8  2  0
  7  9  1  0
  9 10  2  0
  9 11  1  0
  3 12  1  0
  4 13  1  0
  4 14  1  0
  5 15  1  0
  5 16  1  0
  6 17  1  0
  6 18  1  0
 11 19  1  0
M  END
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PDB for HMDB0000225 (Oxoadipic acid)

HEADER    PROTEIN                                 03-SEP-20   NONE
TITLE     NULL                                                        
COMPND    MOLECULE: Oxoadipic acid.mol                                
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   03-SEP-20         0                                  
HETATM    1  C   UNK     0      -3.334   0.385   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0      -2.001  -0.385   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0      -0.667   0.385   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0       0.667  -0.385   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0       2.001   0.385   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0       3.334  -0.385   0.000  0.00  0.00           C+0
HETATM    7  O   UNK     0      -3.334   1.925   0.000  0.00  0.00           O+0
HETATM    8  O   UNK     0      -4.668  -0.385   0.000  0.00  0.00           O+0
HETATM    9  O   UNK     0       3.334  -1.925   0.000  0.00  0.00           O+0
HETATM   10  O   UNK     0       4.668   0.385   0.000  0.00  0.00           O+0
HETATM   11  O   UNK     0      -2.001  -1.925   0.000  0.00  0.00           O+0
CONECT    1    2    7    8                                                  
CONECT    2    1    3   11                                                  
CONECT    3    2    4                                                       
CONECT    4    3    5                                                       
CONECT    5    4    6                                                       
CONECT    6    5    9   10                                                  
CONECT    7    1                                                            
CONECT    8    1                                                            
CONECT    9    6                                                            
CONECT   10    6                                                            
CONECT   11    2                                                            
MASTER        0    0    0    0    0    0    0    0   11    0   20    0
END
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3D PDB for HMDB0000225 (Oxoadipic acid)

COMPND    HMDB0000225
HETATM    1  O1  UNL     1       1.635  -1.565   0.758  1.00  0.00           O  
HETATM    2  C1  UNL     1       2.337  -0.780   0.104  1.00  0.00           C  
HETATM    3  O2  UNL     1       3.717  -0.789   0.348  1.00  0.00           O  
HETATM    4  C2  UNL     1       1.838   0.162  -0.910  1.00  0.00           C  
HETATM    5  C3  UNL     1       0.350   0.098  -0.993  1.00  0.00           C  
HETATM    6  C4  UNL     1      -0.360   0.478   0.261  1.00  0.00           C  
HETATM    7  C5  UNL     1      -1.829   0.380   0.066  1.00  0.00           C  
HETATM    8  O3  UNL     1      -2.257   0.016  -1.052  1.00  0.00           O  
HETATM    9  C6  UNL     1      -2.789   0.689   1.113  1.00  0.00           C  
HETATM   10  O4  UNL     1      -2.368   1.059   2.251  1.00  0.00           O  
HETATM   11  O5  UNL     1      -4.159   0.598   0.925  1.00  0.00           O  
HETATM   12  H1  UNL     1       4.029  -0.524   1.273  1.00  0.00           H  
HETATM   13  H2  UNL     1       2.214   1.183  -0.655  1.00  0.00           H  
HETATM   14  H3  UNL     1       2.271  -0.060  -1.911  1.00  0.00           H  
HETATM   15  H4  UNL     1       0.006   0.764  -1.831  1.00  0.00           H  
HETATM   16  H5  UNL     1       0.094  -0.956  -1.282  1.00  0.00           H  
HETATM   17  H6  UNL     1      -0.171   1.583   0.418  1.00  0.00           H  
HETATM   18  H7  UNL     1      -0.050  -0.107   1.128  1.00  0.00           H  
HETATM   19  H8  UNL     1      -4.510   0.405  -0.010  1.00  0.00           H  
CONECT    1    2    2
CONECT    2    3    4
CONECT    3   12
CONECT    4    5   13   14
CONECT    5    6   15   16
CONECT    6    7   17   18
CONECT    7    8    8    9
CONECT    9   10   10   11
CONECT   11   19
END
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SMILES for HMDB0000225 (Oxoadipic acid)

OC(=O)CCCC(=O)C(O)=O
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INCHI for HMDB0000225 (Oxoadipic acid)

InChI=1S/C6H8O5/c7-4(6(10)11)2-1-3-5(8)9/h1-3H2,(H,8,9)(H,10,11)
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View in JSmolView NMR AssignmentsView Stereo Labels
Synonyms
Chemical FormulaC6H8O5
Average Molecular Weight160.1247
Monoisotopic Molecular Weight160.037173366
IUPAC Name2-oxohexanedioic acid
Traditional Nameoxoadipate
CAS Registry Number3184-35-8
SMILES
OC(=O)CCCC(=O)C(O)=O
InChI Identifier
InChI=1S/C6H8O5/c7-4(6(10)11)2-1-3-5(8)9/h1-3H2,(H,8,9)(H,10,11)
InChI KeyFGSBNBBHOZHUBO-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as medium-chain keto acids and derivatives. These are keto acids with a 6 to 12 carbon atoms long side chain.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassKeto acids and derivatives
Sub ClassMedium-chain keto acids and derivatives
Direct ParentMedium-chain keto acids and derivatives
Alternative Parents
Substituents
  • Medium-chain keto acid
  • Dicarboxylic acid or derivatives
  • Alpha-keto acid
  • Alpha-hydroxy ketone
  • Ketone
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Health effect
Disposition
Biological locationRoute of exposureSource
Process
Naturally occurring process
Role
Indirect biological roleBiological roleIndustrial application
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point127 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Not Available127.0http://allccs.zhulab.cn/database/detail?ID=AllCCS00000012
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Mitochondria
Biospecimen Locations
  • Blood
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified520-970 umol/mmol creatinineChildren (1-13 years old)Female
2-Aminoadipic aciduria		 details
UrineDetected and Quantified62 umol/mmol creatinineAdolescent (13-18 years old)Malealpha-Ketoadipic Aciduria details
UrineDetected and Quantified20-120 umol/mmol creatinineChildren (1-13 years old)Not Specifiedalpha-Aminoadipic aciduria details
UrineDetected and Quantified456 umol/mmol creatinineInfant (0-1 year old)Not Specified
alpha-ketoadipic aciduria		 details
UrineDetected and Quantified135 umol/mmol creatinineNewborn (0-30 days old)Not Specified
alpha-ketoadipic aciduria		 details
Associated Disorders and Diseases
Disease References
2-Ketoadipic acidemia
  1. Chalmers RA, Lawson AM, Watts RW, Tavill AS, Kamerling JP, Hey E, Ogilvie D: D-2-hydroxyglutaric aciduria: case report and biochemical studies. J Inherit Metab Dis. 1980;3(1):11-5. [PubMed:6774165 ]
  2. Peng H, Shinka T, Inoue Y, Mitsubuchi H, Ishimatsu J, Yoshino M, Kuhara T: Asymptomatic alpha-ketoadipic aciduria detected during a pilot study of neonatal urine screening. Acta Paediatr. 1999 Aug;88(8):911-4. [PubMed:10503694 ]
  3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Alpha-aminoadipic aciduria
  1. Gray RG, O'Neill EM, Pollitt RJ: Alpha-aminoadipic aciduria: chemical and enzymatic studies. J Inherit Metab Dis. 1980;2(4):89-92. [PubMed:6796766 ]
2-Aminoadipic aciduria
  1. Danhauser K, Sauer SW, Haack TB, Wieland T, Staufner C, Graf E, Zschocke J, Strom TM, Traub T, Okun JG, Meitinger T, Hoffmann GF, Prokisch H, Kolker S: DHTKD1 mutations cause 2-aminoadipic and 2-oxoadipic aciduria. Am J Hum Genet. 2012 Dec 7;91(6):1082-7. doi: 10.1016/j.ajhg.2012.10.006. Epub 2012 Nov 8. [PubMed:23141293 ]
Associated OMIM IDs
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB003362
KNApSAcK IDC00000770
Chemspider ID70
KEGG Compound IDC00322
BioCyc ID2K-ADIPATE
BiGG ID1485244
Wikipedia LinkNot Available
METLIN ID5234
PubChem Compound71
PDB IDNot Available
ChEBI ID15753
Food Biomarker OntologyNot Available
VMH ID2OXOADP
MarkerDB IDMDB00000107
Good Scents IDNot Available
References
Synthesis ReferenceNelson, Randall B.; Gribble, Gordon W. Preparation of a-ketoadipic acid. Organic Preparations and Procedures International (1973), 5(2), 55-8.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Fiermonte G, Dolce V, Palmieri L, Ventura M, Runswick MJ, Palmieri F, Walker JE: Identification of the human mitochondrial oxodicarboxylate carrier. Bacterial expression, reconstitution, functional characterization, tissue distribution, and chromosomal location. J Biol Chem. 2001 Mar 16;276(11):8225-30. Epub 2000 Nov 16. [PubMed:11083877 ]
  2. Lee SH, Kim SO, Chung BC: Gas chromatographic-mass spectrometric determination of urinary oxoacids using O-(2,3,4,5,6-pentafluorobenzyl)oxime-trimethylsilyl ester derivatization and cation-exchange chromatography. J Chromatogr B Biomed Sci Appl. 1998 Nov 20;719(1-2):1-7. [PubMed:9869358 ]
  3. 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 ]
  4. Barshop BA, Nyhan WL, Naviaux RK, McGowan KA, Friedlander M, Haas RH: Kearns-Sayre syndrome presenting as 2-oxoadipic aciduria. Mol Genet Metab. 2000 Jan;69(1):64-8. [PubMed:10655159 ]
  5. Schulze-Bergkamen A, Okun JG, Spiekerkotter U, Lindner M, Haas D, Kohlmuller D, Mayatepek E, Schulze-Bergkamen H, Greenberg CR, Zschocke J, Hoffmann GF, Kolker S: Quantitative acylcarnitine profiling in peripheral blood mononuclear cells using in vitro loading with palmitic and 2-oxoadipic acids: biochemical confirmation of fatty acid oxidation and organic acid disorders. Pediatr Res. 2005 Nov;58(5):873-80. Epub 2005 Sep 23. [PubMed:16183823 ]

Enzymes

Enzyme Details
1. Dihydrolipoyl dehydrogenase, mitochondrial
General function:
Involved in oxidoreductase activity
Specific function:
Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.
Gene Name:
DLD
Uniprot ID:
P09622
Molecular weight:
54176.91
Enzyme Details
2. Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial
General function:
Involved in transferase activity, transferring nitrogenous groups
Specific function:
Transaminase with broad substrate specificity. Has transaminase activity towards aminoadipate, kynurenine, methionine and glutamate. Shows activity also towards tryptophan, aspartate and hydroxykynurenine. Accepts a variety of oxo-acids as amino-group acceptors, with a preference for 2-oxoglutarate, 2-oxocaproic acid, phenylpyruvate and alpha-oxo-gamma-methiol butyric acid. Can also use glyoxylate as amino-group acceptor (in vitro).
Gene Name:
AADAT
Uniprot ID:
Q8N5Z0
Molecular weight:
47351.17
Reactions
Aminoadipic acid + Oxoglutaric acid → Oxoadipic acid + Glutamic aciddetails
Enzyme Details
3. 2-oxoglutarate dehydrogenase, mitochondrial
General function:
Involved in oxoglutarate dehydrogenase (succinyl-transferring) activity
Specific function:
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3).
Gene Name:
OGDH
Uniprot ID:
Q02218
Molecular weight:
48179.59
Reactions
Oxoadipic acid + Coenzyme A + NAD → Glutaryl-CoA + Carbon dioxide + NADH + Hydrogen Iondetails
Oxoadipic acid + Enzyme N6-(lipoyl)lysine → [Dihydrolipoyllysine-residue succinyltransferase] S-glutaryldihydrolipoyllysine + Carbon dioxidedetails
Enzyme Details
4. Aminoadipate aminotransferase, isoform CRA_b
General function:
Transcription
Specific function:
Not Available
Gene Name:
AADAT
Uniprot ID:
Q4W5N8
Molecular weight:
47351.2
Enzyme Details
5. 2-oxoglutarate dehydrogenase-like, mitochondrial
General function:
Involved in oxoglutarate dehydrogenase (succinyl-transferring) activity
Specific function:
Not Available
Gene Name:
OGDHL
Uniprot ID:
Q9ULD0
Molecular weight:
Not Available
Reactions
Oxoadipic acid + Coenzyme A + NAD → Glutaryl-CoA + Carbon dioxide + NADH + Hydrogen Iondetails
Oxoadipic acid + Enzyme N6-(lipoyl)lysine → [Dihydrolipoyllysine-residue succinyltransferase] S-glutaryldihydrolipoyllysine + Carbon dioxidedetails