Succinic acid
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Identification
- Brand Names
- Irospan 24/6 Kit
- Generic Name
- Succinic acid
- DrugBank Accession Number
- DB00139
- Background
A water-soluble, colorless crystal with an acid taste that is used as a chemical intermediate, in medicine, the manufacture of lacquers, and to make perfume esters. It is also used in foods as a sequestrant, buffer, and a neutralizing agent. (Hawley's Condensed Chemical Dictionary, 12th ed, p1099; McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed, p1851)
- Type
- Small Molecule
- Groups
- Approved, Nutraceutical
- Structure
- Weight
- Average: 118.088
Monoisotopic: 118.02660868 - Chemical Formula
- C4H6O4
- Synonyms
- 1,2-ethanedicarboxylic acid
- Acide butanedioïque
- Acide succinique
- Acidum succinicum
- Amber acid
- Asuccin
- Bernsteinsäure
- Butandisäure
- Butanedioic acid
- Butanedionic acid
- Dihydrofumaric acid
- Ethylenesuccinic acid
- Spirit of amber
- Succinic acid
- External IDs
- E363
Pharmacology
- Indication
For nutritional supplementation, also for treating dietary shortage or imbalance
Reduce drug development failure ratesBuild, train, & validate machine-learning modelswith evidence-based and structured datasets.Build, train, & validate predictive machine-learning models with structured datasets.- Associated Therapies
- Contraindications & Blackbox Warnings
- Prevent Adverse Drug Events TodayTap into our Clinical API for life-saving information on contraindications & blackbox warnings, population restrictions, harmful risks, & more.Avoid life-threatening adverse drug events with our Clinical API
- Pharmacodynamics
Not Available
- Mechanism of action
Succinate is an essential component of the Krebs or citric acid cycle and serves an electron donor in the production of fumaric acid and FADH2. It also has been shown to be a good "natural" antibiotic because of its relative acidic or caustic nature (high concentrations can even cause burns). Succinate supplements have been shown to help reduce the effects of hangovers by activating the degradation of acetaldehyde - a toxic byproduct of alcohol metabolism - into CO2 and H2O through aerobic metabolism. Succinic acid has been shown to stimulate neural system recovery and bolster the immune system. Claims have also been made that it boosts awareness, concentration and reflexes.
Target Actions Organism ASuccinate-semialdehyde dehydrogenase, mitochondrial inhibitorHumans USuccinate receptor 1 Not Available Humans - Absorption
Not Available
- Volume of distribution
Not Available
- Protein binding
Not Available
- Metabolism
- Not Available
- Route of elimination
Not Available
- Half-life
Not Available
- Clearance
Not Available
- Adverse Effects
- Improve decision support & research outcomesWith structured adverse effects data, including: blackbox warnings, adverse reactions, warning & precautions, & incidence rates. View sample adverse effects data in our new Data Library!Improve decision support & research outcomes with our structured adverse effects data.
- Toxicity
Oral rat LD50: 2260 mg/kg
- Pathways
- Pharmacogenomic Effects/ADRs
- Not Available
Interactions
- Drug Interactions
- This information should not be interpreted without the help of a healthcare provider. If you believe you are experiencing an interaction, contact a healthcare provider immediately. The absence of an interaction does not necessarily mean no interactions exist.
Drug Interaction Integrate drug-drug
interactions in your softwareAcetylsalicylic acid The excretion of Succinic acid can be decreased when combined with Acetylsalicylic acid. Acyclovir The excretion of Succinic acid can be decreased when combined with Acyclovir. Aminohippuric acid The excretion of Succinic acid can be decreased when combined with Aminohippuric acid. Apalutamide The excretion of Succinic acid can be decreased when combined with Apalutamide. Ataluren The excretion of Succinic acid can be decreased when combined with Ataluren. - Food Interactions
- No interactions found.
Products
- Drug product information from 10+ global regionsOur datasets provide approved product information including:dosage, form, labeller, route of administration, and marketing period.Access drug product information from over 10 global regions.
- Product Images
- Mixture Products
Name Ingredients Dosage Route Labeller Marketing Start Marketing End Region Image Ferrex 150 Plus Succinic acid (50 mg/1) + Ascorbic acid (50 mg/1) + Iron (150 mg/1) Capsule Oral Breckenridge Pharmaceutical, Inc. 2009-09-30 2012-12-31 US - Unapproved/Other Products
Name Ingredients Dosage Route Labeller Marketing Start Marketing End Region Image Ferrex 150 Forte Plus Succinic acid (50 mg/1) + Calcium ascorbate dihydrate (60 mg/1) + Calcium threonate (0.8 mg/1) + Cyanocobalamin (25 ug/1) + Folic acid (1 mg/1) + Iron (150 mg/1) Capsule Oral Breckenridge Pharmaceutical, Inc. 2008-08-01 2012-09-30 US Ferrex 150 Plus Succinic acid (50 mg/1) + Ascorbic acid (50 mg/1) + Iron (150 mg/1) Capsule Oral Breckenridge Pharmaceutical, Inc. 2009-09-30 2012-12-31 US Ferrex 28 Succinic acid (150 mg/1) + Succinic acid (150 mg/1) + Ascorbic acid (200 mg/1) + Cyanocobalamin (10 ug/1) + Ferrous fumarate (81 mg/1) + Folic acid (1 mg/1) + Iron (70 mg/1) Kit Oral Breckenridge Pharmaceutical, Inc. 2009-06-26 2011-07-31 US Ferrex 28 Succinic acid (150 mg/1) + Succinic acid (150 mg/1) + Ascorbic acid (200 mg/1) + Cyanocobalamin (10 ug/1) + Ferrous fumarate (81 mg/1) + Folic acid (1 mg/1) + Iron (70 mg/1) Kit Oral Breckenridge Pharmaceutical, Inc. 2009-06-26 2011-07-31 US Irospan 24/6 Succinic acid (150 mg/1) + Biotin (300 ug/1) + Biotin (300 ug/1) + Calcium carbonate (100 mg/1) + Cyanocobalamin (10 ug/1) + Cyanocobalamin (10 ug/1) + Ferrous bisglycinate (65 mg/1) + Folic acid (1 mg/1) + Folic acid (1 mg/1) + Calcium formate (155 mg/1) + Iron Dextran (65 mg/1) + Niacin (25 mg/1) + Niacin (25 mg/1) + Calcium pantothenate (300 ug/1) + Calcium pantothenate (7 mg/1) + Pyridoxine hydrochloride (30 mg/1) + Pyridoxine hydrochloride (30 mg/1) + Riboflavin sodium (5 mg/1) + Riboflavin (5 mg/1) + Sodium ascorbate (100 mg/1) + Sodium ascorbate (100 mg/1) + Thiamine hydrochloride (5 mg/1) + Thiamine hydrochloride (5 mg/1) Kit; Tablet; Tablet, film coated Oral WOMENS CHOICE PHARMACEUTICALS LLC 2011-04-15 Not applicable US
Categories
- Drug Categories
- Chemical TaxonomyProvided by Classyfire
- Description
- This compound belongs to the class of organic compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups.
- Kingdom
- Organic compounds
- Super Class
- Organic acids and derivatives
- Class
- Carboxylic acids and derivatives
- Sub Class
- Dicarboxylic acids and derivatives
- Direct Parent
- Dicarboxylic acids and derivatives
- Alternative Parents
- Fatty acids and conjugates / Carboxylic acids / Organic oxides / Hydrocarbon derivatives / Carbonyl compounds
- Substituents
- Aliphatic acyclic compound / Carbonyl group / Carboxylic acid / Dicarboxylic acid or derivatives / Fatty acid / Hydrocarbon derivative / Organic oxide / Organic oxygen compound / Organooxygen compound
- Molecular Framework
- Aliphatic acyclic compounds
- External Descriptors
- C4-dicarboxylic acid, alpha,omega-dicarboxylic acid (CHEBI:15741) / Dicarboxylic acids (LMFA01170043)
- Affected organisms
- Humans and other mammals
Chemical Identifiers
- UNII
- AB6MNQ6J6L
- CAS number
- 110-15-6
- InChI Key
- KDYFGRWQOYBRFD-UHFFFAOYSA-N
- InChI
- InChI=1S/C4H6O4/c5-3(6)1-2-4(7)8/h1-2H2,(H,5,6)(H,7,8)
- IUPAC Name
- butanedioic acid
- SMILES
- OC(=O)CCC(O)=O
References
- Synthesis Reference
Michael Diamantoglou, Gerhard Meyer, "Process for the production of water-insoluble fibers of cellulose monoesters of maleic acid, succinic acid and phthalic acid, having an extremely high absorbability for water and physiological liquids." U.S. Patent US4734239, issued April, 1941.
US4734239- General References
- Not Available
- External Links
- Human Metabolome Database
- HMDB0000254
- KEGG Compound
- C00042
- PubChem Compound
- 1110
- PubChem Substance
- 46505859
- ChemSpider
- 1078
- BindingDB
- 26121
- 37255
- ChEBI
- 15741
- ChEMBL
- CHEMBL576
- ZINC
- ZINC000000895030
- Therapeutic Targets Database
- DAP000545
- PharmGKB
- PA164747739
- PDBe Ligand
- SIN
- Wikipedia
- Succinic_acid
- PDB Entries
- 1abz / 1cze / 1d4d / 1e5h / 1f8i / 1gp6 / 1h2z / 1h6c / 1hg0 / 1hqj … show 315 more
- MSDS
- Download (71.8 KB)
Clinical Trials
- Clinical Trials
Clinical Trial & Rare Diseases Add-on Data Package
Explore 4,000+ rare diseases, orphan drugs & condition pairs, clinical trial why stopped data, & more. Preview package Phase Status Purpose Conditions Count Start Date Why Stopped 100+ additional columns Unlock 175K+ rows when you subscribe.View sample dataNot Available Completed Not Available Stroke, Acute 1 somestatus stop reason just information to hide 4 Recruiting Treatment Iron Deficiency Anemia of Pregnancy / Iron Storage Disease 1 somestatus stop reason just information to hide 4 Terminated Treatment Anemia 1 somestatus stop reason just information to hide 3 Completed Treatment Cognitive Dysfunctions 1 somestatus stop reason just information to hide 3 Completed Treatment Diabetic Neuropathies 1 somestatus stop reason just information to hide
Pharmacoeconomics
- Manufacturers
- Not Available
- Packagers
- Breckenridge Pharmaceuticals
- Contract Pharm
- J T Baker
- KV Pharmaceutical Co.
- Murfreesboro Pharmaceutical Nursing Supply
- National Vitamin Company
- Physicians Total Care Inc.
- PruGen Inc. Pharmaceuticals
- Rasi Laboratories Inc.
- Seton Pharmaceuticals LLC
- Ther-Rx Corp.
- Dosage Forms
Form Route Strength Capsule Oral Kit Oral Tablet Oral Kit; tablet; tablet, film coated Oral - Prices
Unit description Cost Unit Succinic acid cryst reagent 0.15USD g DrugBank does not sell nor buy drugs. Pricing information is supplied for informational purposes only.- Patents
- Not Available
Properties
- State
- Solid
- Experimental Properties
Property Value Source melting point (°C) 188 °C PhysProp boiling point (°C) 235 dec °C PhysProp water solubility 8.32E+004 mg/L (at 25 °C) YALKOWSKY,SH & HE,Y (2003) logP -0.59 HANSCH,C ET AL. (1995) logS -0.2 ADME Research, USCD pKa 4.21 (at 25 °C) DEAN,JA (1987) - Predicted Properties
Property Value Source Water Solubility 211.0 mg/mL ALOGPS logP -0.53 ALOGPS logP -0.4 Chemaxon logS 0.25 ALOGPS pKa (Strongest Acidic) 3.55 Chemaxon Physiological Charge -2 Chemaxon Hydrogen Acceptor Count 4 Chemaxon Hydrogen Donor Count 2 Chemaxon Polar Surface Area 74.6 Å2 Chemaxon Rotatable Bond Count 3 Chemaxon Refractivity 23.54 m3·mol-1 Chemaxon Polarizability 10.14 Å3 Chemaxon Number of Rings 0 Chemaxon Bioavailability 1 Chemaxon Rule of Five Yes Chemaxon Ghose Filter No Chemaxon Veber's Rule No Chemaxon MDDR-like Rule No Chemaxon - Predicted ADMET Features
Property Value Probability Human Intestinal Absorption + 0.668 Blood Brain Barrier + 0.869 Caco-2 permeable - 0.648 P-glycoprotein substrate Non-substrate 0.7618 P-glycoprotein inhibitor I Non-inhibitor 0.9796 P-glycoprotein inhibitor II Non-inhibitor 0.9701 Renal organic cation transporter Non-inhibitor 0.9513 CYP450 2C9 substrate Non-substrate 0.8535 CYP450 2D6 substrate Non-substrate 0.9161 CYP450 3A4 substrate Non-substrate 0.7794 CYP450 1A2 substrate Non-inhibitor 0.8848 CYP450 2C9 inhibitor Non-inhibitor 0.9759 CYP450 2D6 inhibitor Non-inhibitor 0.9713 CYP450 2C19 inhibitor Non-inhibitor 0.9856 CYP450 3A4 inhibitor Non-inhibitor 0.9679 CYP450 inhibitory promiscuity Low CYP Inhibitory Promiscuity 0.9951 Ames test Non AMES toxic 0.9587 Carcinogenicity Non-carcinogens 0.7929 Biodegradation Ready biodegradable 0.9399 Rat acute toxicity 1.6866 LD50, mol/kg Not applicable hERG inhibition (predictor I) Weak inhibitor 0.9622 hERG inhibition (predictor II) Non-inhibitor 0.9771
Spectra
- Mass Spec (NIST)
- Download (9.46 KB)
- Spectra
- Chromatographic Properties
Collision Cross Sections (CCS)
Adduct CCS Value (Å2) Source type Source [M-H]- 118.6949342 predictedDarkChem Lite v0.1.0 [M-H]- 118.7485342 predictedDarkChem Lite v0.1.0 [M-H]- 118.8169342 predictedDarkChem Lite v0.1.0 [M-H]- 120.237 predictedDeepCCS 1.0 (2019) [M-H]- 118.6949342 predictedDarkChem Lite v0.1.0 [M-H]- 118.7485342 predictedDarkChem Lite v0.1.0 [M-H]- 118.8169342 predictedDarkChem Lite v0.1.0 [M-H]- 120.237 predictedDeepCCS 1.0 (2019) [M+H]+ 123.013504 predictedDeepCCS 1.0 (2019) [M+H]+ 123.013504 predictedDeepCCS 1.0 (2019) [M+Na]+ 131.53387 predictedDeepCCS 1.0 (2019) [M+Na]+ 131.53387 predictedDeepCCS 1.0 (2019)
Targets
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Yes
- Actions
- Inhibitor
- General Function
- Catalyzes one step in the degradation of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA)
- Specific Function
- identical protein binding
- Gene Name
- ALDH5A1
- Uniprot ID
- P51649
- Uniprot Name
- Succinate-semialdehyde dehydrogenase, mitochondrial
- Molecular Weight
- 57214.23 Da
References
- Yogeeswari P, Sriram D, Vaigundaragavendran J: The GABA shunt: an attractive and potential therapeutic target in the treatment of epileptic disorders. Curr Drug Metab. 2005 Apr;6(2):127-39. [Article]
- Popov VN, Eprintsev AT, Fedorin DN, Fomenko OIu, Igamberdiev AU: [Role of transamination in the mobilization of respiratory substrates in germinating seeds of castor oil plants]. Prikl Biokhim Mikrobiol. 2007 May-Jun;43(3):376-81. [Article]
- Wang C, Zhang HB, Wang LH, Zhang LH: Succinic semialdehyde couples stress response to quorum-sensing signal decay in Agrobacterium tumefaciens. Mol Microbiol. 2006 Oct;62(1):45-56. Epub 2006 Aug 30. [Article]
- Ahn SJ, Yang CH, Cooksey DA: Pseudomonas putida 06909 genes expressed during colonization on mycelial surfaces and phenotypic characterization of mutants. J Appl Microbiol. 2007 Jul;103(1):120-32. [Article]
- Chiribau CB, Mihasan M, Ganas P, Igloi GL, Artenie V, Brandsch R: Final steps in the catabolism of nicotine. FEBS J. 2006 Apr;273(7):1528-36. [Article]
- Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. [Article]
- Zhou Y, Zhang Y, Zhao D, Yu X, Shen X, Zhou Y, Wang S, Qiu Y, Chen Y, Zhu F: TTD: Therapeutic Target Database describing target druggability information. Nucleic Acids Res. 2024 Jan 5;52(D1):D1465-D1477. doi: 10.1093/nar/gkad751. [Article]
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- General Function
- G protein-coupled receptor for succinate able to mediate signaling through Gq/GNAQ or Gi/GNAI second messengers depending on the cell type and the processes regulated (By similarity) (PubMed:15141213, PubMed:23770096, PubMed:34133934). Succinate-SUCNR1 signaling serves as a link between metabolic stress, inflammation and energy homeostasis (PubMed:18820681, PubMed:34133934). In macrophages, plays a range of immune-regulatory roles. During inflammation, succinate-SUCNR1 signaling may act as an anti-inflammatory mediator or boost inflammation depending on the inflammatory status of cells (By similarity). Hyperpolarizes M2 macrophages versus M1 phenotype through Gq signaling by regulating the transcription of genes involved in immune function (PubMed:34133934). In activated M1 macrophages, plays a pro-inflammatory role in response to LPS (By similarity). Expressed in dendritic cells, where it is involved in the sensing of immunological danger and enhances immunity. Mediates succinate triggered intracelleular calcium mobilization, induces migratory responses and acts in synergy with Toll-like receptor ligands for the production of proinflammatory cytokines as well as an enhancement of antigen-specific activation of helper T cells (PubMed:18820681). In the small intestine, mediates the activation of tuft cells by dietary succinate and triggers type 2 immunity (By similarity). In adipocytes, plays an important role in the control of energy metabolism. In response to succinate, controls leptin expression in an AMPK-JNK-CEBPA-dependent as well as circadian clock-regulated manner (By similarity). In muscle tissue, is expressed in non-muscle cells and coordinates muscle remodeling in response to the succinate produced during exercise training in a paracrine manner (By similarity). In retina, acts as a mediator of vessel growth during retinal development. In response to succinate, regulates the production of angiogenic factors, including VEGF, by retinal ganglion neurons (By similarity)
- Specific Function
- G protein-coupled receptor activity
- Gene Name
- SUCNR1
- Uniprot ID
- Q9BXA5
- Uniprot Name
- Succinate receptor 1
- Molecular Weight
- 38697.395 Da
References
- Macaulay IC, Tijssen MR, Thijssen-Timmer DC, Gusnanto A, Steward M, Burns P, Langford CF, Ellis PD, Dudbridge F, Zwaginga JJ, Watkins NA, van der Schoot CE, Ouwehand WH: Comparative gene expression profiling of in vitro differentiated megakaryocytes and erythroblasts identifies novel activatory and inhibitory platelet membrane proteins. Blood. 2007 Apr 15;109(8):3260-9. Epub 2006 Dec 27. [Article]
Enzymes
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Substrate
- General Function
- Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (PubMed:9533030). SDH also oxidizes malate to the non-canonical enol form of oxaloacetate, enol-oxaloacetate (By similarity). Enol-oxaloacetate, which is a potent inhibitor of the succinate dehydrogenase activity, is further isomerized into keto-oxaloacetate (By similarity)
- Specific Function
- electron transfer activity
- Gene Name
- SDHC
- Uniprot ID
- Q99643
- Uniprot Name
- Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
- Molecular Weight
- 18610.03 Da
References
- Rutter J, Winge DR, Schiffman JD: Succinate dehydrogenase - Assembly, regulation and role in human disease. Mitochondrion. 2010 Jun;10(4):393-401. doi: 10.1016/j.mito.2010.03.001. Epub 2010 Mar 10. [Article]
- Bayley JP, Devilee P, Taschner PE: The SDH mutation database: an online resource for succinate dehydrogenase sequence variants involved in pheochromocytoma, paraganglioma and mitochondrial complex II deficiency. BMC Med Genet. 2005 Nov 16;6:39. [Article]
- Szeto SS, Reinke SN, Sykes BD, Lemire BD: Ubiquinone-binding site mutations in the Saccharomyces cerevisiae succinate dehydrogenase generate superoxide and lead to the accumulation of succinate. J Biol Chem. 2007 Sep 14;282(37):27518-26. Epub 2007 Jul 18. [Article]
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Substrate
- General Function
- Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (Probable) (PubMed:24781757). SDH also oxidizes malate to the non-canonical enol form of oxaloacetate, enol-oxaloacetate (By similarity). Enol-oxaloacetate, which is a potent inhibitor of the succinate dehydrogenase activity, is further isomerized into keto-oxaloacetate (By similarity). Can act as a tumor suppressor (PubMed:20484225)
- Specific Function
- electron transfer activity
- Gene Name
- SDHA
- Uniprot ID
- P31040
- Uniprot Name
- Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
- Molecular Weight
- 72690.975 Da
References
- Maklashina E, Iverson TM, Sher Y, Kotlyar V, Andrell J, Mirza O, Hudson JM, Armstrong FA, Rothery RA, Weiner JH, Cecchini G: Fumarate reductase and succinate oxidase activity of Escherichia coli complex II homologs are perturbed differently by mutation of the flavin binding domain. J Biol Chem. 2006 Apr 21;281(16):11357-65. Epub 2006 Feb 15. [Article]
- Takeo S, Kokaze A, Ng CS, Mizuchi D, Watanabe JI, Tanabe K, Kojima S, Kita K: Succinate dehydrogenase in Plasmodium falciparum mitochondria: molecular characterization of the SDHA and SDHB genes for the catalytic subunits, the flavoprotein (Fp) and iron-sulfur (Ip) subunits. Mol Biochem Parasitol. 2000 Apr 15;107(2):191-205. [Article]
- Ackrell BA: Cytopathies involving mitochondrial complex II. Mol Aspects Med. 2002 Oct;23(5):369-84. [Article]
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Product of
- General Function
- GTP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit
- Specific Function
- ATP binding
- Gene Name
- SUCLG2
- Uniprot ID
- Q96I99
- Uniprot Name
- Succinate--CoA ligase [GDP-forming] subunit beta, mitochondrial
- Molecular Weight
- 46510.215 Da
References
- Majumdar R, Guest JR, Bridger WA: Functional consequences of substitution of the active site (phospho)histidine residue of Escherichia coli succinyl-CoA synthetase. Biochim Biophys Acta. 1991 Jan 8;1076(1):86-90. doi: 10.1016/0167-4838(91)90223-m. [Article]
- Van Hove JL, Saenz MS, Thomas JA, Gallagher RC, Lovell MA, Fenton LZ, Shanske S, Myers SM, Wanders RJ, Ruiter J, Turkenburg M, Waterham HR: Succinyl-CoA ligase deficiency: a mitochondrial hepatoencephalomyopathy. Pediatr Res. 2010 Aug;68(2):159-64. doi: 10.1203/00006450-201011001-00310. [Article]
- Kind
- Protein group
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Product of
- General Function
- Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins
- Specific Function
- identical protein binding
Components:
References
- Gorres KL, Raines RT: Prolyl 4-hydroxylase. Crit Rev Biochem Mol Biol. 2010 Apr;45(2):106-24. doi: 10.3109/10409231003627991. [Article]
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Product of
- General Function
- Multifunctional enzyme that catalyzes a series of essential post-translational modifications on Lys residues in procollagen (PubMed:11956192, PubMed:12475640, PubMed:18298658, PubMed:18834968, PubMed:30089812). Plays a redundant role in catalyzing the formation of hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens (PubMed:11956192, PubMed:12475640, PubMed:18298658, PubMed:18834968, PubMed:30089812, PubMed:9582318, PubMed:9724729). Plays a redundant role in catalyzing the transfer of galactose onto hydroxylysine groups, giving rise to galactosyl 5-hydroxylysine (PubMed:12475640, PubMed:18298658, PubMed:18834968, PubMed:30089812). Has an essential role by catalyzing the subsequent transfer of glucose moieties, giving rise to 1,2-glucosylgalactosyl-5-hydroxylysine residues (PubMed:10934207, PubMed:11896059, PubMed:11956192, PubMed:12475640, PubMed:18298658, PubMed:18834968, PubMed:30089812). Catalyzes hydroxylation and glycosylation of Lys residues in the MBL1 collagen-like domain, giving rise to hydroxylysine and 1,2-glucosylgalactosyl-5-hydroxylysine residues (PubMed:25419660). Essential for normal biosynthesis and secretion of type IV collagens (Probable) (PubMed:18834968). Essential for normal formation of basement membranes (By similarity)
- Specific Function
- iron ion binding
- Gene Name
- PLOD3
- Uniprot ID
- O60568
- Uniprot Name
- Multifunctional procollagen lysine hydroxylase and glycosyltransferase LH3
- Molecular Weight
- 84784.505 Da
References
- Guo HF, Cho EJ, Devkota AK, Chen Y, Russell W, Phillips GN Jr, Yamauchi M, Dalby KN, Kurie JM: A scalable lysyl hydroxylase 2 expression system and luciferase-based enzymatic activity assay. Arch Biochem Biophys. 2017 Mar 15;618:45-51. doi: 10.1016/j.abb.2017.02.003. Epub 2017 Feb 20. [Article]
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Product of
- General Function
- Basement membrane-associated chondroitin sulfate proteoglycan (CSPG). Has prolyl 3-hydroxylase activity catalyzing the post-translational formation of 3-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens, especially types IV and V. May be involved in the secretory pathway of cells. Has growth suppressive activity in fibroblasts
- Specific Function
- iron ion binding
- Gene Name
- P3H1
- Uniprot ID
- Q32P28
- Uniprot Name
- Prolyl 3-hydroxylase 1
- Molecular Weight
- 83393.195 Da
References
- Place TL, Domann FE: Prolyl-hydroxylase 3: Evolving Roles for an Ancient Signaling Protein. Hypoxia (Auckl). 2013 Oct 1;2013(1):13-17. doi: 10.2147/HP.S50091. [Article]
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Product of
- General Function
- Prolyl 3-hydroxylase that catalyzes the post-translational formation of 3-hydroxyproline on collagens (PubMed:18487197). Contributes to proline 3-hydroxylation of collagen COL4A1 and COL1A1 in tendons, the eye sclera and in the eye lens capsule (By similarity). Has high activity with the type IV collagen COL4A1, and lower activity with COL1A1 (PubMed:18487197). Catalyzes hydroxylation of the first Pro in Gly-Pro-Hyp sequences where Hyp is 4-hydroxyproline (PubMed:18487197). Has no activity on substrates that lack 4-hydroxyproline in the third position (PubMed:18487197)
- Specific Function
- iron ion binding
- Gene Name
- P3H2
- Uniprot ID
- Q8IVL5
- Uniprot Name
- Prolyl 3-hydroxylase 2
- Molecular Weight
- 80983.685 Da
References
- Place TL, Domann FE: Prolyl-hydroxylase 3: Evolving Roles for an Ancient Signaling Protein. Hypoxia (Auckl). 2013 Oct 1;2013(1):13-17. doi: 10.2147/HP.S50091. [Article]
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Product of
- General Function
- Part of a complex composed of PLOD1, P3H3 and P3H4 that catalyzes hydroxylation of lysine residues in collagen alpha chains and is required for normal assembly and cross-linkling of collagen fibrils. Required for normal hydroxylation of lysine residues in type I collagen chains in skin, bone, tendon, aorta and cornea. Required for normal skin stability via its role in hydroxylation of lysine residues in collagen alpha chains and in collagen fibril assembly. Apparently not required for normal prolyl 3-hydroxylation on collagen chains, possibly because it functions redundantly with other prolyl 3-hydroxylases
- Specific Function
- iron ion binding
- Gene Name
- P3H3
- Uniprot ID
- Q8IVL6
- Uniprot Name
- Prolyl 3-hydroxylase 3
- Molecular Weight
- 81835.705 Da
References
- Place TL, Domann FE: Prolyl-hydroxylase 3: Evolving Roles for an Ancient Signaling Protein. Hypoxia (Auckl). 2013 Oct 1;2013(1):13-17. doi: 10.2147/HP.S50091. [Article]
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Product of
- General Function
- Key enzyme for ketone body catabolism. Catalyzes the first, rate-limiting step of ketone body utilization in extrahepatic tissues, by transferring coenzyme A (CoA) from a donor thiolester species (succinyl-CoA) to an acceptor carboxylate (acetoacetate), and produces acetoacetyl-CoA. Acetoacetyl-CoA is further metabolized by acetoacetyl-CoA thiolase into two acetyl-CoA molecules which enter the citric acid cycle for energy production (PubMed:10964512). Forms a dimeric enzyme where both of the subunits are able to form enzyme-CoA thiolester intermediates, but only one subunit is competent to transfer the CoA moiety to the acceptor carboxylate (3-oxo acid) and produce a new acyl-CoA. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate (By similarity)
- Specific Function
- succinyl-CoA
- Gene Name
- OXCT1
- Uniprot ID
- P55809
- Uniprot Name
- Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial
- Molecular Weight
- 56157.175 Da
References
- Coros AM, Swenson L, Wolodko WT, Fraser ME: Structure of the CoA transferase from pig heart to 1.7 A resolution. Acta Crystallogr D Biol Crystallogr. 2004 Oct;60(Pt 10):1717-25. Epub 2004 Sep 23. [Article]
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Product of
- General Function
- Specifically hydroxylates an Asp or Asn residue in certain epidermal growth factor-like (EGF) domains of a number of proteins
- Specific Function
- calcium ion binding
- Gene Name
- ASPH
- Uniprot ID
- Q12797
- Uniprot Name
- Aspartyl/asparaginyl beta-hydroxylase
- Molecular Weight
- 85862.095 Da
References
- Lahousse SA, Carter JJ, Xu XJ, Wands JR, de la Monte SM: Differential growth factor regulation of aspartyl-(asparaginyl)-beta-hydroxylase family genes in SH-Sy5y human neuroblastoma cells. BMC Cell Biol. 2006 Dec 7;7:41. doi: 10.1186/1471-2121-7-41. [Article]
Transporters
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Substrate
- General Function
- Functions as an organic anion/dicarboxylate exchanger that couples organic anion uptake indirectly to the sodium gradient (PubMed:14586168, PubMed:15644426, PubMed:15846473, PubMed:16455804, PubMed:31553721). Transports organic anions such as estrone 3-sulfate (E1S) and urate in exchange for dicarboxylates such as glutarate or ketoglutarate (2-oxoglutarate) (PubMed:14586168, PubMed:15846473, PubMed:15864504, PubMed:22108572, PubMed:23832370). Plays an important role in the excretion of endogenous and exogenous organic anions, especially from the kidney and the brain (PubMed:11306713, PubMed:14586168, PubMed:15846473). E1S transport is pH- and chloride-dependent and may also involve E1S/cGMP exchange (PubMed:26377792). Responsible for the transport of prostaglandin E2 (PGE2) and prostaglandin F2(alpha) (PGF2(alpha)) in the basolateral side of the renal tubule (PubMed:11907186). Involved in the transport of neuroactive tryptophan metabolites kynurenate and xanthurenate (PubMed:22108572, PubMed:23832370). Functions as a biopterin transporters involved in the uptake and the secretion of coenzymes tetrahydrobiopterin (BH4), dihydrobiopterin (BH2) and sepiapterin to urine, thereby determining baseline levels of blood biopterins (PubMed:28534121). May be involved in the basolateral transport of steviol, a metabolite of the popular sugar substitute stevioside (PubMed:15644426). May participate in the detoxification/ renal excretion of drugs and xenobiotics, such as the histamine H(2)-receptor antagonists fexofenadine and cimetidine, the antibiotic benzylpenicillin (PCG), the anionic herbicide 2,4-dichloro-phenoxyacetate (2,4-D), the diagnostic agent p-aminohippurate (PAH), the antiviral acyclovir (ACV), and the mycotoxin ochratoxin (OTA), by transporting these exogenous organic anions across the cell membrane in exchange for dicarboxylates such as 2-oxoglutarate (PubMed:11669456, PubMed:15846473, PubMed:16455804). Contributes to the renal uptake of potent uremic toxins (indoxyl sulfate (IS), indole acetate (IA), hippurate/N-benzoylglycine (HA) and 3-carboxy-4-methyl-5-propyl-2-furanpropionate (CMPF)), pravastatin, PCG, E1S and dehydroepiandrosterone sulfate (DHEAS), and is partly involved in the renal uptake of temocaprilat (an angiotensin-converting enzyme (ACE) inhibitor) (PubMed:14675047). May contribute to the release of cortisol in the adrenals (PubMed:15864504). Involved in one of the detoxification systems on the choroid plexus (CP), removes substrates such as E1S or taurocholate (TC), PCG, 2,4-D and PAH, from the cerebrospinal fluid (CSF) to the blood for eventual excretion in urine and bile (By similarity). Also contributes to the uptake of several other organic compounds such as the prostanoids prostaglandin E(2) and prostaglandin F(2-alpha), L-carnitine, and the therapeutic drugs allopurinol, 6-mercaptopurine (6-MP) and 5-fluorouracil (5-FU) (By similarity). Mediates the transport of PAH, PCG, and the statins pravastatin and pitavastatin, from the cerebrum into the blood circulation across the blood-brain barrier (BBB). In summary, plays a role in the efflux of drugs and xenobiotics, helping reduce their undesired toxicological effects on the body (By similarity)
- Specific Function
- organic anion transmembrane transporter activity
- Gene Name
- SLC22A8
- Uniprot ID
- Q8TCC7
- Uniprot Name
- Organic anion transporter 3
- Molecular Weight
- 59855.585 Da
References
- Cha SH, Sekine T, Fukushima JI, Kanai Y, Kobayashi Y, Goya T, Endou H: Identification and characterization of human organic anion transporter 3 expressing predominantly in the kidney. Mol Pharmacol. 2001 May;59(5):1277-86. [Article]
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Ligand
- General Function
- Catalyzes the electroneutral exchange or flux of physiologically important metabolites such as dicarboxylates (malonate, malate, succinate), inorganic sulfur-containing anions, and phosphate, across mitochondrial inner membrane (PubMed:29211846). Plays an important role in gluconeogenesis, fatty acid metabolism, urea synthesis, and sulfur metabolism, particularly in liver, by supplying the substrates for the different metabolic processes. Regulates fatty acid release from adipocytes, and contributes to systemic insulin sensitivity (By similarity)
- Specific Function
- antiporter activity
- Gene Name
- SLC25A10
- Uniprot ID
- Q9UBX3
- Uniprot Name
- Mitochondrial dicarboxylate carrier
- Molecular Weight
- 31282.21 Da
References
- Ventura FV, Ruiter J, Ijlst L, de Almeida IT, Wanders RJ: Differential inhibitory effect of long-chain acyl-CoA esters on succinate and glutamate transport into rat liver mitochondria and its possible implications for long-chain fatty acid oxidation defects. Mol Genet Metab. 2005 Nov;86(3):344-52. Epub 2005 Sep 19. [Article]
- Mizuarai S, Miki S, Araki H, Takahashi K, Kotani H: Identification of dicarboxylate carrier Slc25a10 as malate transporter in de novo fatty acid synthesis. J Biol Chem. 2005 Sep 16;280(37):32434-41. Epub 2005 Jul 15. [Article]
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Inhibitor
- General Function
- Sodium:sulfate symporter that mediates sulfate reabsorption in the kidney and small intestine (PubMed:11161786). Can also mediate the transport of selenate and thiosulfate (By similarity)
- Specific Function
- monoatomic anion
- Gene Name
- SLC13A1
- Uniprot ID
- Q9BZW2
- Uniprot Name
- Solute carrier family 13 member 1
- Molecular Weight
- 66133.62 Da
References
- Lee A, Beck L, Markovich D: The human renal sodium sulfate cotransporter (SLC13A1; hNaSi-1) cDNA and gene: organization, chromosomal localization, and functional characterization. Genomics. 2000 Dec 15;70(3):354-63. [Article]
- Markovich D: Na+-sulfate cotransporter SLC13A1. Pflugers Arch. 2014 Jan;466(1):131-7. doi: 10.1007/s00424-013-1388-8. Epub 2013 Nov 6. [Article]
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Substrate
- General Function
- Low-affinity sodium-dicarboxylate cotransporter, that mediates the entry of citric acid cycle intermediates, such as succinate, citrate, fumarate and alpha-ketoglutarate (2-oxoglutarate) into the small intestine and renal proximal tubule (PubMed:10894787, PubMed:8967342, PubMed:9668069). Transports the dicarboxylate into the cell with a probable stoichiometry of 3 Na(+) for 1 divalent dicarboxylate, rendering the process electrogenic (PubMed:10894787, PubMed:8967342, PubMed:9668069). Citrate is transported in protonated form as a divalent anion, rather than the trivalent form which is normally found in blood (PubMed:10894787). Has a critical role in renal dicarboxylate transport (By similarity)
- Specific Function
- alpha-ketoglutarate transmembrane transporter activity
- Gene Name
- SLC13A2
- Uniprot ID
- Q13183
- Uniprot Name
- Solute carrier family 13 member 2
- Molecular Weight
- 64409.495 Da
References
- Pajor AM: Molecular properties of the SLC13 family of dicarboxylate and sulfate transporters. Pflugers Arch. 2006 Feb;451(5):597-605. doi: 10.1007/s00424-005-1487-2. Epub 2005 Oct 7. [Article]
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Substrate
- General Function
- High-affinity sodium-dicarboxylate cotransporter that accepts a range of substrates with 4-6 carbon atoms, such as the citric acid cycle intermediates succinate and alpha-ketoglutarate (2-oxoglutarate), as well as other compounds including N-acetyl-L-aspartate (PubMed:10794676, PubMed:10992006, PubMed:15561973, PubMed:17356845, PubMed:17426067, PubMed:24247155, PubMed:30635937). Transports the dicarboxylate into the cell with a probable stoichiometry of 3 Na(+) for 1 divalent dicarboxylate, rendering the process electrogenic (PubMed:10794676, PubMed:10992006). Can transport citrate in a Na(+)-dependent manner, recognizing the divalent form of citrate rather than the trivalent form which is normally found in blood (PubMed:10794676)
- Specific Function
- alpha-ketoglutarate transmembrane transporter activity
- Gene Name
- SLC13A3
- Uniprot ID
- Q8WWT9
- Uniprot Name
- Na(+)/dicarboxylate cotransporter 3
- Molecular Weight
- 66840.355 Da
References
- Pajor AM: Molecular properties of the SLC13 family of dicarboxylate and sulfate transporters. Pflugers Arch. 2006 Feb;451(5):597-605. doi: 10.1007/s00424-005-1487-2. Epub 2005 Oct 7. [Article]
Drug created at June 13, 2005 13:24 / Updated at August 26, 2024 19:23