T3DB: 2-Hydroxyadipic acid

Identification Taxonomy Biological Properties Physical Properties Toxicity Profile Spectra Concentrations Links References Gene Regulation XML

Record Information

Version

2.0

Creation Date

2014-08-29 06:08:10 UTC

Update Date

2018-03-21 17:46:12 UTC

Accession Number

T3D4258

Identification

Common Name

2-Hydroxyadipic acid

Class

Small Molecule

Description

2-Hydroxyadipic acid is a hydroxy-dicarboxylic acid formed by the reduction of 2-ketoadipic acid. A deficiency of 2-ketoadipic dehydrogenase causes 2-ketoadipic acidemia or 2-oxoadipic acidemia (OMIM: 245130), a genetic disorder characterized by accumulation and excretion of 2-hydroxyadipic acid (with 2-ketoadipic and 2-aminoadipic) (OMMBID - The Online Metabolic and Molecular Bases of Inherited Disease, CH.95). When present in sufficiently high levels, 2-hydroxyadipic 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. Chronically high levels of 2-hydroxyadipic acid are associated with at least three inborn errors of metabolism including 2-oxoadipic acidemia, 2-aminoadipic aciduria, and 2-oxoadipic aciduria. 2-Hydroxyadipic 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 abnormalities, kidney abnormalities, liver damage, 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.

Compound Type

Animal Toxin
Food Toxin
Metabolite
Natural Compound
Organic Compound

Chemical Structure

MOL SDF PDB SMILES InChI

Synonyms

Synonym
2,3,4-Trideoxyhexarate
2,3,4-Trideoxyhexaric acid
2-Hydroxy-adipate
2-Hydroxy-adipic acid
2-Hydroxy-hexanedioate
2-Hydroxy-hexanedioic acid
2-Hydroxyadipate
2-Hydroxyhexanedioate
2-Hydroxyhexanedioic acid
a-Hydroxyadipate
a-Hydroxyadipic acid
alpha-Hydroxyadipate
alpha-Hydroxyadipic acid
DL-2-Hydroxyadipate
DL-2-Hydroxyadipic acid

Chemical Formula

C₆H₁₀O₅

Average Molecular Mass

162.141 g/mol

Monoisotopic Mass

162.053 g/mol

CAS Registry Number

18294-85-4

IUPAC Name

2-hydroxyhexanedioic acid

Traditional Name

α-hydroxyadipic acid

SMILES

OC(CCCC(O)=O)C(O)=O

InChI Identifier

InChI=1/C6H10O5/c7-4(6(10)11)2-1-3-5(8)9/h4,7H,1-3H2,(H,8,9)(H,10,11)

InChI Key

InChIKey=OTTXIFWBPRRYOG-UHFFFAOYNA-N

Chemical Taxonomy

Description

belongs to the class of organic compounds known as medium-chain fatty acids. These are fatty acids with an aliphatic tail that contains between 4 and 12 carbon atoms.

Kingdom

Organic compounds

Super Class

Lipids and lipid-like molecules

Class

Fatty Acyls

Sub Class

Fatty acids and conjugates

Direct Parent

Medium-chain fatty acids

Alternative Parents

Substituents

Medium-chain fatty acid
Hydroxy fatty acid
Monosaccharide
Hydroxy acid
Dicarboxylic acid or derivatives
Alpha-hydroxy acid
Secondary alcohol
Carboxylic acid
Carboxylic acid derivative
Hydrocarbon derivative
Organic oxygen compound
Organooxygen compound
Alcohol
Carbonyl group
Organic oxide
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

2-hydroxydicarboxylic acid (CHEBI:17023 )
Dicarboxylic acids (LMFA01170049 )

Biological Properties

Status

Detected and Not Quantified

Origin

Endogenous

Cellular Locations

Cytoplasm
Extracellular

Biofluid Locations

Not Available

Tissue Locations

Not Available

Pathways

Name	SMPDB Link	KEGG Link
2-aminoadipic 2-oxoadipic aciduria	SMP00719	Not Available

Applications

Not Available

Biological Roles

Not Available

Chemical Roles

Not Available

Physical Properties

State

Solid

Appearance

White powder.

Experimental Properties

Property	Value
Melting Point	Not Available
Boiling Point	Not Available
Solubility	Not Available
LogP	Not Available

Predicted Properties

Property	Value	Source
Water Solubility	70.3 g/L	ALOGPS
logP	-0.79	ALOGPS
logP	-0.38	ChemAxon
logS	-0.36	ALOGPS
pKa (Strongest Acidic)	3.44	ChemAxon
pKa (Strongest Basic)	-3.8	ChemAxon
Physiological Charge	-2	ChemAxon
Hydrogen Acceptor Count	5	ChemAxon
Hydrogen Donor Count	3	ChemAxon
Polar Surface Area	94.83 Å²	ChemAxon
Rotatable Bond Count	5	ChemAxon
Refractivity	34.23 m³·mol⁻¹	ChemAxon
Polarizability	14.88 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	1	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	Yes	ChemAxon
Veber's Rule	Yes	ChemAxon
MDDR-like Rule	Yes	ChemAxon

Spectra

Spectrum Type	Description	Splash Key	Deposition Date	View
GC-MS	GC-MS Spectrum - GC-MS (3 TMS)	splash10-00c0-2920000000-14e371ab410997076c08	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-00c0-2920000000-14e371ab410997076c08	2017-09-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-00xu-9200000000-a5e63603281ceddefa05	2017-09-01	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (3 TMS) - 70eV, Positive	splash10-01w0-9273000000-da104d83dee59e6410a4	2017-10-06	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_3_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 10V, Negative (Annotated)	splash10-03di-2900000000-00b8bf461ba454c702f9	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 25V, Negative (Annotated)	splash10-0ab9-9000000000-cbfcbe2f1e74c7fa8544	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 40V, Negative (Annotated)	splash10-052f-9000000000-1a3f1223d56568a37ca5	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Negative	splash10-03di-3900000000-2f9a1134097c3e464cfa	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 40V, Negative	splash10-0a4i-9000000000-38b55b37770b02b19786	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 20V, Negative	splash10-0600-9200000000-2e8f145f65b692f6de62	2021-09-20	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-0002-1900000000-c97aa5c57fc34910b084	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-00kb-9800000000-8b422e7bd31b7715f936	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-0596-9000000000-145898d967788e6a4370	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-03dl-0900000000-b8d680487ea735b08660	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-014m-5900000000-2748f3664e3329b9721b	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0aba-9000000000-7bd0454dd4e607bf327d	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-002b-7900000000-ffa4a95821301025a0c9	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-05fs-9200000000-91faff169b9ab7ca25fb	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-0a4l-9000000000-f90638e9886a94d83928	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-0007-5900000000-6ce0e78d81edfe6486c2	2021-09-23	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-00kb-9500000000-e93f435014ff8c6374e9	2021-09-23	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0007-9000000000-af7c0163f8f183203d48	2021-09-23	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, H₂O, experimental)	Not Available	2012-12-04	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	Not Available	2021-09-29	View Spectrum
2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, H₂O, experimental)	Not Available	2012-12-05	View Spectrum

Toxicity Profile

Route of Exposure

Not Available

Mechanism of Toxicity

2-Hydroxyadipic acid is a hydroxy-dicarboxylic acid formed by the reduction of 2-ketoadipic acid. Deficiency of 2-ketoadipic dehydrogenase causes 2-ketoadipic acidemia, a condition characterized by accumulation and excretion of 2-hydroxyadipic acid (with 2-ketoadipic and 2-aminoadipic) probably without adverse phenotypic effects.

Metabolism

Not Available

Toxicity Values

Not Available

Lethal Dose

Not Available

Carcinogenicity (IARC Classification)

No indication of carcinogenicity to humans (not listed by IARC).

Uses/Sources

This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation.

Minimum Risk Level

Not Available

Health Effects

Chronically high levels of 2-hydroxyadipic acid are associated with at least 2 inborn errors of metabolism including: : 2-aminoadipic and 2-oxoadipic aciduria.

Symptoms

Not Available

Treatment

Not Available

Normal Concentrations

Not Available

Abnormal Concentrations

Not Available

External Links

DrugBank ID

Not Available

HMDB ID

HMDB00321

PubChem Compound ID

193530

ChEMBL ID

Not Available

ChemSpider ID

167943

KEGG ID

C02360

UniProt ID

Not Available

OMIM ID

ChEBI ID

17023

BioCyc ID

Not Available

CTD ID

Not Available

Stitch ID

Not Available

PDB ID

Not Available

ACToR ID

Not Available

Wikipedia Link

Not Available

References

Synthesis Reference

Griot, Rudolf. a-Hydroxyadipic acid. Helvetica Chimica Acta (1958), 41 2236-41.

MSDS

Not Available

General References

Svendsen JS, Whist JE, Sydnes LK: Absolute configuration of 3-hydroxyadipic acid in human urine. J Chromatogr. 1985 Jan 11;337(1):9-19. [3980660 ]
Sewell AC, Heil M, Podebrad F, Mosandl A: Chiral compounds in metabolism: a look in the molecular mirror. Eur J Pediatr. 1998 Mar;157(3):185-91. [9537483 ]
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. [8087979 ]
Inoue Y, Shinka T, Ohse M, Kuhara T: Differential chemical diagnosis of primary hyperoxaluria type II. Highly sensitive analysis of optical isomers of glyceric acid by GC/MS as diastereoisomeric derivatives. J Chromatogr B Analyt Technol Biomed Life Sci. 2005 Aug 25;823(1):2-6. Epub 2005 Apr 25. [16055048 ]

Gene Regulation

Up-Regulated Genes

Not Available

Down-Regulated Genes

Not Available

2-Hydroxyadipic acid (T3D4258)

Structure for T3D4258: 2-Hydroxyadipic acid