Human Metabolome Database: Showing metabocard for Isocitric acid (HMDB0000193)

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Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (7) Show 7 proteins XML

enzymes (7) Show 7 proteins

Record Information

Version

4.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2020-02-26 21:21:55 UTC

HMDB ID

HMDB0000193

Secondary Accession Numbers

HMDB00193

Metabolite Identification

Common Name

Isocitric acid

Description

Isocitric acid, also known as isocitrate or i-cit, belongs to the class of organic compounds known as tricarboxylic acids and derivatives. These are carboxylic acids containing exactly three carboxyl groups. Isocitric acid is an extremely weak basic (essentially neutral) compound (based on its pKa). Click on genes, proteins and metabolites below to link to respective articles. An isocitric acid value higher than this may be indicative of fruit juice adulteration. Isocitric acid exists in all living species, ranging from bacteria to humans. Isocitrate is formed from citrate with the help of the enzyme aconitase, and is acted upon by isocitrate dehydrogenase. Outside of the human body, isocitric acid has been detected, but not quantified in, several different foods, such as redcurrants, wild celeries, gooseberries, soursops, and apples. This could make isocitric acid a potential biomarker for the consumption of these foods. Salts and esters of isocitric acid are known as isocitrates. In authentic orange juice, for example, the ratio of citric acid to D-isocitric acid is usually less than 130. Isocitric acid is a structural isomer of citric acid. The isocitrate anion is a substrate of the citric acid cycle. Isocitric acid is commonly used as a marker to detect the authenticity and quality of fruit products, most often citrus juices.

Structure

MOL SDF PDB SMILES InChI

Synonyms

Value	Source
1-Hydroxypropane-1,2,3-tricarboxylic acid	ChEBI
1-Hydroxytricarballylic acid	ChEBI
1-Hydroxypropane-1,2,3-tricarboxylate	Generator
1-Hydroxytricarballylate	Generator
Isocitrate	Generator
1-Hydroxy-1,2,3-propanetricarboxylate	HMDB
1-Hydroxy-1,2,3-propanetricarboxylic acid	HMDB
3-Carboxy-2,3-dideoxy-1-hydroxypropan-1,2,3-tricarboxylate	HMDB
3-Carboxy-2,3-dideoxy-1-hydroxypropan-1,2,3-tricarboxylic acid	HMDB
3-Carboxy-2,3-dideoxy-pentarate	HMDB
3-Carboxy-2,3-dideoxy-pentaric acid	HMDB
D-Isocitrate	HMDB
I-cit	HMDB
Threo-D(S)-iso-citrate	HMDB
Threo-DS-isocitrate	HMDB
Isocitric acid, sodium salt	HMDB
Isocitric acid, trisodium salt	HMDB
Isocitric acid, disodium salt	HMDB
Isocitric acid, (11)C-labeled	HMDB
Isocitric acid, calcium salt	HMDB
Isocitric acid, potassium salt	HMDB

Chemical Formula

C₆H₈O₇

Average Molecular Weight

192.1235

Monoisotopic Molecular Weight

192.02700261

IUPAC Name

1-hydroxypropane-1,2,3-tricarboxylic acid

Traditional Name

isocitric acid

CAS Registry Number

320-77-4

SMILES

OC(C(CC(O)=O)C(O)=O)C(O)=O

InChI Identifier

InChI=1S/C6H8O7/c7-3(8)1-2(5(10)11)4(9)6(12)13/h2,4,9H,1H2,(H,7,8)(H,10,11)(H,12,13)

InChI Key

ODBLHEXUDAPZAU-UHFFFAOYSA-N

Chemical Taxonomy

Description

belongs to the class of organic compounds known as tricarboxylic acids and derivatives. These are carboxylic acids containing exactly three carboxyl groups.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Tricarboxylic acids and derivatives

Direct Parent

Tricarboxylic acids and derivatives

Alternative Parents

Substituents

Tricarboxylic acid or derivatives
Beta-hydroxy acid
Monosaccharide
Hydroxy acid
Alpha-hydroxy acid
Secondary alcohol
Carboxylic acid
Organic oxygen compound
Organic oxide
Hydrocarbon derivative
Organooxygen compound
Carbonyl group
Alcohol
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

tricarboxylic acid (CHEBI:30887 )

Ontology

Physiological effect

Health effect:

Health condition:

Anoxia

Disposition

Route of exposure:

Enteral:

Ingestion

Source:

Biological:

Plant

Biological location:

Organ and components:

Endocrine gland:

Adrenal gland

Biofluid and excreta:

Subcellular:

Process

Naturally occurring process:

Biological process:

Biochemical pathway:

Physical Properties

State

Solid

Experimental Properties

Property	Value	Reference
Melting Point	162 - 165 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	466 mg/mL	Not Available
LogP	Not Available	Not Available

Predicted Properties

Property	Value	Source
Water Solubility	52.5 g/L	ALOGPS
logP	-0.35	ALOGPS
logP	-1.4	ChemAxon
logS	-0.56	ALOGPS
pKa (Strongest Acidic)	3.07	ChemAxon
pKa (Strongest Basic)	-4	ChemAxon
Physiological Charge	-3	ChemAxon
Hydrogen Acceptor Count	7	ChemAxon
Hydrogen Donor Count	4	ChemAxon
Polar Surface Area	132.13 Å²	ChemAxon
Rotatable Bond Count	5	ChemAxon
Refractivity	35.72 m³·mol⁻¹	ChemAxon
Polarizability	15.55 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Spectra

Spectrum Type	Description	Splash Key	View
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (4 TMS)	splash10-0002-1952000000-7c34751686a6bdb54cab	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (4 TMS)	splash10-0002-0963000000-459b45ff9246b5f55914	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-0002-0942000000-61204d02a28e43d21607	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (4 TMS)	splash10-00di-9420000000-4e1a59fe735855d52ce2	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (4 TMS)	splash10-00ea-3793000000-84858fa896697420275f	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0002-1952000000-7c34751686a6bdb54cab	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0002-0963000000-459b45ff9246b5f55914	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-00di-9420000000-4e1a59fe735855d52ce2	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-00ea-3793000000-84858fa896697420275f	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-00ba-7900000000-96194d87d52b155cf1b9	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (4 TMS) - 70eV, Positive	splash10-02vi-7029500000-2bb65fc897e48cc21750	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)	splash10-0udi-1900000000-8e1185b0504ea1547715	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)	splash10-05fr-7900000000-0855c1ceb168d556b7f2	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)	splash10-0bu1-9500000000-140b46d0f1dc8fcd9e49	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-00fu-0940000000-033739944b190c47c6a7	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-053r-1900000000-89ff897aa220452bfa16	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-00di-0900000000-36aeb28d857c9039938b	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-03di-1900000000-53373dc0755caa954310	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-0006-0901310000-883764bf7b686ca7fd17	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-03di-0900000000-66d03405eeb126c6ad9d	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-00di-0900000000-cdad17a11f419421c787	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-0udi-0009000000-d3440c84a488b3e22212	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative	splash10-0006-0900000000-3aa263561a7a49df7d3d	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative	splash10-0006-1900000000-3636f87026e4206a1638	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative	splash10-01w3-9600000000-ab1a2b9a29528a623d0b	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative	splash10-0079-9000000000-3c33cefeb87f85efd5de	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative	splash10-059m-9000000000-34775db8bbc008018633	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-0006-0900000000-3aa263561a7a49df7d3d	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-0006-1900000000-3636f87026e4206a1638	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-01w3-9600000000-ab1a2b9a29528a623d0b	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-004j-0900000000-a75a4b486df1552d6d93	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-0gla-4900000000-d04fec4218cafd6195df	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-0fk9-8900000000-4e5fbbb415df9fbc8f31	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-00ke-1900000000-c364bae4640e201c366d	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-0v4j-4900000000-d00f263a75673b7981c7	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0pk9-9200000000-156693398229f972d717	Spectrum
1D NMR	1H NMR Spectrum	Not Available	Spectrum
2D NMR	[1H,13C] 2D NMR Spectrum	Not Available	Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Mitochondria
Peroxisome

Biospecimen Locations

Blood
Cellular Cytoplasm
Cerebrospinal Fluid (CSF)
Feces
Saliva
Semen
Urine

Tissue Locations

Adrenal Gland

Pathways

Name	SMPDB/Pathwhiz	KEGG
Citric Acid Cycle
Congenital lactic acidosis		Not Available
Fumarase deficiency		Not Available
Mitochondrial complex II deficiency		Not Available
2-ketoglutarate dehydrogenase complex deficiency		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	6.0 (0.0-10.0) uM	Adult (>18 years old)	Both	Normal	8412012	details
Cellular Cytoplasm	Detected and Quantified	38 (23-53) uM	Adult (>18 years old)	Both	Normal	8579834	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	10.0 +/- 9.0 uM	Adult (>18 years old)	Not Specified	Normal	8412012	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	29808030	details
Saliva	Detected and Quantified	4.17 +/- 1.75 uM	Adult (>18 years old)	Female	Normal	23857558	details
Saliva	Detected and Quantified	0.988 +/- 0.531 uM	Adult (>18 years old)	Not Specified	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	0.294 +/- 0.267 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Both	Normal	Zerihun T. Dame, ...	details
Saliva	Detected and Quantified	0.616 +/- 0.351 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	0.616 +/- 0.351 uM	Adult (>18 years old)	Not Specified	Normal	Sugimoto et al. (...	details
Semen	Detected and Quantified	400 - 1500 uM	Adult (>18 years old)	Both	Normal	8115279	details
Urine	Detected but not Quantified		Adult (>18 years old)	Both	Normal	25733631	details
Urine	Detected and Quantified	52.403 +/- 28.756 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Mordechai, Hien, ...	details
Urine	Detected and Quantified	56.8(19.4-119.1) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected but not Quantified		Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected but not Quantified		Adult (>18 years old)	Both	Normal	21389975	details
Urine	Detected and Quantified	30-57 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	27012787	details
Urine	Detected but not Quantified		Adult (>18 years old)	Both	Normal	28157703	details
Urine	Detected and Quantified	94.3 (29.8-154.4) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	8087979	details
Urine	Detected and Quantified	120.1 (101.1-265.3) umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	8087979	details
Urine	Detected and Quantified	53.7 (31.9-83.1) umol/mmol creatinine	Children (1-13 years old)	Both	Normal	8087979	details
Urine	Detected and Quantified	45.0 (18.0-69.9) umol/mmol creatinine	Children (1-13 years old)	Both	Normal	8087979	details
Urine	Detected and Quantified	38.9 (16.0-118.0) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	8087979	details
Urine	Detected and Quantified	19.0 (5.0-34.0) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Geigy Scientific ...	details
Urine	Detected and Quantified	7.7 (5.0-12.0) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	Geigy Scientific ...	details
Urine	Detected and Quantified	24.52 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	Shaykhutdinov RA,...	details
Urine	Detected and Quantified	58 (36-84) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	2702744	details
Urine	Detected and Quantified	46.9 (20.4-89.2) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	19.08 (5.26 - 42.76) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	657545	details
Urine	Detected and Quantified	22.0 +/- 13.8 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	15992788	details
Urine	Detected and Quantified	27-43 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	27012787	details
Urine	Detected and Quantified	14.7 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	21388201	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Cellular Cytoplasm	Detected and Quantified	15 (14.8-15.2) uM	Adult (>18 years old)	Both	Anoxia	8579834	details
Saliva	Detected and Quantified	3.75 +/- 2.89 uM	Adult (>18 years old)	Male	Alzheimer's disease	23857558	details
Saliva	Detected and Quantified	3.28 +/- 1.43 uM	Adult (>18 years old)	Male	Frontotemporal lobe dementia	23857558	details
Saliva	Detected and Quantified	3.98 +/- 0.15 uM	Adult (>18 years old)	Both	Lewy body disease	23857558	details
Urine	Detected and Quantified	90.008 +/- 97.639 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Mordechai, Hien, ...	details
Urine	Detected but not Quantified		Adult (>18 years old)	Both	Bladder cancer	28157703	details
Urine	Detected but not Quantified		Adult (>18 years old)	Both	Autosomal dominant polycystic kidney disease (ADPKD)	21389975	details

Associated Disorders and Diseases

Disease References

Anoxia
Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed:8579834 ]
Alzheimer's disease
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Frontotemporal dementia
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Lewy body disease
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Autosomal dominant polycystic kidney disease
Gronwald W, Klein MS, Zeltner R, Schulze BD, Reinhold SW, Deutschmann M, Immervoll AK, Boger CA, Banas B, Eckardt KU, Oefner PJ: Detection of autosomal dominant polycystic kidney disease by NMR spectroscopic fingerprinting of urine. Kidney Int. 2011 Jun;79(11):1244-53. doi: 10.1038/ki.2011.30. Epub 2011 Mar 9. [PubMed:21389975 ]
Eosinophilic esophagitis
(). Mordechai, Hien, and David S. Wishart. .

Associated OMIM IDs

104300 (Alzheimer's disease)
600274 (Frontotemporal dementia)
601313 (Autosomal dominant polycystic kidney disease)
610247 (Eosinophilic esophagitis)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

BiGG ID

Wikipedia Link

METLIN ID

PubChem Compound

PDB ID

Not Available

ChEBI ID

30887

Food Biomarker Ontology

Not Available

VMH ID

Not Available

References

Synthesis Reference

Finogenova, T. V.; Kamzolova, S. V.; Dedyukhina, E. G.; Shishkanova, N. V.; Il'chenko, A. P.; Morgunov, I. G.; Chernyavskaya, O. G.; Sokolov, A. P. Biosynthesis of citric and isocitric acids from ethanol by mutant Yarrowia lipolytica N 1 under continuous cultivation. Applied Microbiology and Biotechnology (2002), 59(4-5), 493-500.

Material Safety Data Sheet (MSDS)

Download (PDF)

General References

Stromme JH, Borud O, Moe PJ: Fatal lactic acidosis in a newborn attributable to a congenital defect of pyruvate dehydrogenase. Pediatr Res. 1976 Jan;10(1):62-6. [PubMed:813176 ]
Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed:8579834 ]
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 ]
Sutor DJ, Percival JM, Doonan S: Isolation and identification of some urinary inhibitors of calcium phosphate formation. Clin Chim Acta. 1978 Oct 16;89(2):273-8. [PubMed:213213 ]
Ebeling K, Ruckhaberle KE, Bilek K: [Studies on the recording of cytostatic effects on organ cultures of squamous cell carcinoma of the uterine cervix]. Zentralbl Gynakol. 1977;99(20):1249-59. [PubMed:595962 ]
Sutor DJ, Percival JM, Doonan S: Urinary inhibitor of the formation of calcium oxalate. Br J Urol. 1979 Aug;51(4):253-5. [PubMed:223711 ]
Hennequin C, Lalanne V, Daudon M, Lacour B, Drueke T: A new approach to studying inhibitors of calcium oxalate crystal growth. Urol Res. 1993 Mar;21(2):101-8. [PubMed:8389069 ]
Kavanagh JP: Isocitric and citric acid in human prostatic and seminal fluid: implications for prostatic metabolism and secretion. Prostate. 1994;24(3):139-42. [PubMed:8115279 ]
Mikosha AS, Monissarenko VP, Bychkovskaia LA: [Properties of adrenocortical isocitrate dehydrogenase]. Vopr Med Khim. 1981 Nov-Dec;27(6):736-9. [PubMed:7336646 ]
Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. [PubMed:8412012 ]
Sutor DJ, Percival JM: The estimation of D-isocitric acid in urine using isocitrate dehydrogenase. Clin Chim Acta. 1978 Jun;86(2):223-5. [PubMed:657545 ]

Enzymes

Enzyme Details

1. Aconitate hydratase, mitochondrial

General function:: Involved in metabolic process
Specific function:: Catalyzes the isomerization of citrate to isocitrate via cis-aconitate (By similarity).
Gene Name:: ACO2
Uniprot ID:: Q99798
Molecular weight:: 85424.745

Reactions

Citric acid → Isocitric acid	details
Isocitric acid → cis-Aconitic acid + Water	details

Enzyme Details

2. Cytoplasmic aconitate hydratase

General function:: Involved in metabolic process
Specific function:: Iron sensor. Binds a 4Fe-4S cluster and functions as aconitase when cellular iron levels are high. Functions as mRNA binding protein that regulates uptake, sequestration and utilization of iron when cellular iron levels are low. Binds to iron-responsive elements (IRES) in target mRNA species when iron levels are low. Binding of a 4Fe-4S cluster precludes RNA binding. Catalyzes the isomerization of citrate to isocitrate via cis-aconitate (By similarity).
Gene Name:: ACO1
Uniprot ID:: P21399
Molecular weight:: 98398.14

Reactions

Citric acid → Isocitric acid	details
Isocitric acid → cis-Aconitic acid + Water	details

Enzyme Details

3. Isocitrate dehydrogenase [NADP], mitochondrial

General function:: Involved in magnesium ion binding
Specific function:: Plays a role in intermediary metabolism and energy production. It may tightly associate or interact with the pyruvate dehydrogenase complex.
Gene Name:: IDH2
Uniprot ID:: P48735
Molecular weight:: 50908.915

Reactions

Isocitric acid + NADP → Oxoglutaric acid + CO(2) + NADPH	details
Isocitric acid + NADP → Oxoglutaric acid + Carbon dioxide + NADPH + Hydrogen Ion	details
Isocitric acid + NADP → Oxalosuccinic acid + NADPH + Hydrogen Ion	details

Enzyme Details

4. Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial

General function:: Involved in magnesium ion binding
Specific function:: Not Available
Gene Name:: IDH3B
Uniprot ID:: O43837
Molecular weight:: 42183.39

Reactions

Isocitric acid + NAD → Oxoglutaric acid + CO(2) + NADH	details
Isocitric acid + NAD → Oxoglutaric acid + Carbon dioxide + NADH + Hydrogen Ion	details

Enzyme Details

5. Isocitrate dehydrogenase [NADP] cytoplasmic

General function:: Involved in magnesium ion binding
Specific function:: Not Available
Gene Name:: IDH1
Uniprot ID:: O75874
Molecular weight:: 46659.005

Reactions

Isocitric acid + NADP → Oxoglutaric acid + CO(2) + NADPH	details
Isocitric acid + NADP → Oxoglutaric acid + Carbon dioxide + NADPH + Hydrogen Ion	details
Isocitric acid + NADP → Oxalosuccinic acid + NADPH + Hydrogen Ion	details

Enzyme Details

6. Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial

General function:: Involved in magnesium ion binding
Specific function:: Not Available
Gene Name:: IDH3A
Uniprot ID:: P50213
Molecular weight:: 39591.365

Reactions

Isocitric acid + NAD → Oxoglutaric acid + CO(2) + NADH	details
Isocitric acid + NAD → Oxoglutaric acid + Carbon dioxide + NADH + Hydrogen Ion	details

Enzyme Details

7. Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial

General function:: Involved in magnesium ion binding
Specific function:: Not Available
Gene Name:: IDH3G
Uniprot ID:: P51553
Molecular weight:: 42793.97

Reactions

Isocitric acid + NAD → Oxoglutaric acid + CO(2) + NADH	details
Isocitric acid + NAD → Oxoglutaric acid + Carbon dioxide + NADH + Hydrogen Ion	details

Showing metabocard for Isocitric acid (HMDB0000193)

Structure for HMDB0000193 (Isocitric acid)

Enzymes

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