Identification

Name
Hydroxocobalamin
Accession Number
DB00200  (APRD01022)
Type
Small Molecule
Groups
Approved
Description

Hydroxocobalamin, also known as vitamin B12a and hydroxycobalamin, is an injectable form of vitamin B 12 that has been used therapeutically to treat vitamin B 12 deficiency. Other uses include cyanide poisoning, Leber's optic atrophy, and toxic amblyopia.

Structure
Thumb
Synonyms
  • Hydroxocobalamin anhydrous
  • Hydroxocobalamine
  • Hydroxocobalaminum anhydrous
  • Hydroxycobalamin
  • OH-Cbl
  • Vitamin B-12b
Product Ingredients
IngredientUNIICASInChI Key
Hydroxocobalamin acetateS535M27N3Q22465-48-1XVRCTDQUXAWPBW-FBTIOKFQSA-K
Prescription Products
NameDosageStrengthRouteLabellerMarketing StartMarketing End
CyanokitPowder, for solution5 gIntravenousSerb s.a.2012-10-19Not applicableCanada
CyanokitInjection, powder, lyophilized, for solution5 g/250mLIntravenousMeridian Medical Technologies, Inc.2011-10-01Not applicableUs
CyanokitInjection, powder, for solution5 gIntravenousSerb Sa2007-11-23Not applicableEu
CyanokitPowder, for solution2.5 gIntravenousEmd Serono, A Division Of Emd Inc., Canada2011-04-072014-09-30Canada
CyanokitInjection, powder, lyophilized, for solution2.5 g/100mLIntravenousMeridian Medical Technologies , Inc.2009-08-312013-10-31Us
CyanokitInjection, powder, for solution2.5 gIntravenousSerb Sa2007-11-23Not applicableEu
Hydro Cobex Inj 1000mcg/mlLiquid1 mgIntramuscularKripps Pharmacy Ltd.1979-12-31Not applicableCanada
Hydroxocobalamin Inj 1000mcg/mlLiquid1 mgIntramuscularE L Stickley And Co Ltd.1968-12-311996-09-09Canada
Hydroxy Cobal Inj 1000mcg/mlLiquid1 mgIntramuscularMerit Pharmaceuticals1984-12-312016-08-22Canada
Generic Prescription Products
NameDosageStrengthRouteLabellerMarketing StartMarketing End
CyanokitInjection, powder, lyophilized, for solution2.5 g/100mLIntravenousDey, L.P.2007-03-232011-09-30Us
HydroxocobalaminInjection, solution1000 ug/1mLIntramuscularA-S Medication Solutions2010-11-12Not applicableUs
HydroxocobalaminInjection, solution1000 ug/1mLIntramuscularActavis Pharma Company2010-11-12Not applicableUs
HydroxocobalaminInjection, solution1000 ug/1mLIntramuscularRemedy Repack2013-12-112015-05-29Us
Unapproved/Other Products
NameIngredientsDosageRouteLabellerMarketing StartMarketing End
DermaNICHydroxocobalamin (15 ug/1) + Acetylcysteine zinc (69.5 mg/1) + Chromium nicotinate (0.57 mg/1) + Ferrous cysteine glycinate (1.5 mg/1) + Folic Acid (500 ug/1) + Inositol nicotinate (328 mg/1) + Niacin (1.5 mg/1) + Nicotinamide (498 mg/1)TabletOralAllegis Pharmaceuticals, LLC2014-02-032016-12-01Us
Escavite DHydroxocobalamin (6 ug/1) + Ascorbic acid (100 mg/1) + Beta carotene (2500 [iU]/1) + Biotin (45 ug/1) + Cholecalciferol (600 [iU]/1) + Cupric oxide (2 mg/1) + DL-alpha-Tocopherol (30 [iU]/1) + Ferrous cysteine glycinate (6 mg/1) + Folic Acid (400 ug/1) + Magnesium oxide (60 mg/1) + Niacin (20 mg/1) + Calcium pantothenate (10 mg/1) + Pyridoxine hydrochloride (2 mg/1) + Riboflavin (1.7 mg/1) + Sodium fluoride (0.25 mg/1) + Thiamine hydrochloride (1.5 mg/1) + Zinc oxide (15 mg/1)Tablet, chewableOralGm Pharmaceuticals2014-03-03Not applicableUs
International/Other Brands
Alpha-Redisol (Merck) / Hydrocobamine (Nycomed)
Categories
UNII
Q40X8H422O
CAS number
13422-51-0
Weight
Average: 1346.3551
Monoisotopic: 1345.567070949
Chemical Formula
C62H89CoN13O15P
InChI Key
YOZNUFWCRFCGIH-WZHZPDAFSA-K
InChI
InChI=1S/C62H90N13O14P.Co.H2O/c1-29-20-39-40(21-30(29)2)75(28-70-39)57-52(84)53(41(27-76)87-57)89-90(85,86)88-31(3)26-69-49(83)18-19-59(8)37(22-46(66)80)56-62(11)61(10,25-48(68)82)36(14-17-45(65)79)51(74-62)33(5)55-60(9,24-47(67)81)34(12-15-43(63)77)38(71-55)23-42-58(6,7)35(13-16-44(64)78)50(72-42)32(4)54(59)73-56;;/h20-21,23,28,31,34-37,41,52-53,56-57,76,84H,12-19,22,24-27H2,1-11H3,(H15,63,64,65,66,67,68,69,71,72,73,74,77,78,79,80,81,82,83,85,86);;1H2/q;+3;/p-3/t31-,34-,35-,36-,37+,41-,52-,53-,56-,57+,59-,60+,61+,62+;;/m1../s1
IUPAC Name
(10S,12R,13S,17R,23R,24R,25R,30S,35S,36S,40S,41S,42R,46R)-30,35,40-tris(2-carbamoylethyl)-24,36,41-tris(carbamoylmethyl)-1,46-dihydroxy-12-(hydroxymethyl)-5,6,17,23,28,31,31,36,38,41,42-undecamethyl-15,20-dioxo-11,14,16-trioxa-2lambda5,9,19,26,43lambda5,44lambda5,45lambda5-heptaaza-15lambda5-phospha-1-cobaltadodecacyclo[27.14.1.1^{1,34}.1^{2,9}.1^{10,13}.0^{1,26}.0^{3,8}.0^{23,27}.0^{25,42}.0^{32,44}.0^{39,43}.0^{37,45}]heptatetraconta-2(47),3,5,7,27,29(44),32,34(45),37,39(43)-decaene-2,43,44,45-tetrakis(ylium)-1,1,1-triuid-15-olate
SMILES
[N+]1=2[Co-3]345([N+]6=C7[C@H]([C@@](CC(=O)N)(C)[C@@]6([C@@]6(N3C(=C(C)C3=[N+]4C(C(C)(C)[C@@H]3CCC(=O)N)=CC3=[N+]5C(=C7C)[C@@](CC(=O)N)([C@@H]3CCC(=O)N)C)[C@@](C)([C@H]6CC(=O)N)CCC(NC[C@@H](C)OP(=O)(O[C@@H]3[C@H](O[C@H](N(C4=CC(=C(C=C14)C)C)C=2)[C@@H]3O)CO)[O-])=O)[H])C)CCC(=O)N)O[H]

Pharmacology

Indication

For treatment of pernicious anemia and the prevention and treatment of vitamin B12 deficiency arising from alcoholism, malabsorption, tapeworm infestation, celiac, hyperthyroidism, hepatic-biliary tract disease, persistent diarrhea, ileal resection, pancreatic cancer, renal disease, prolonged stress, vegan diets, macrobiotic diets or other restrictive diets. Also for the treatment of known or suspected cyanide poisoning.

Associated Conditions
Pharmacodynamics

Hydroxocobalamin is a synthetic, injectable form of Vitamin B12. Hydroxocobalamin is actually a precursor of two cofactors or vitamins (Vitamin B12 and Methylcobalamin) which are involved in various biological systems in man. Vitamin B12 is required for the conversion of methylmalonate to succinate. Deficiency of this enzyme could therefore interfere with the production of lipoprotein in myelin sheath tissue and so give rise to neurological lesions. The second cofactor, Methylcobalamin, is necessary for the conversion of homocysteine to methionine which is essential for the metabolism of folic acid. Deficiency of tetrahydrafolate leads to reduced synthesis of thymidylate resulting in reduced synthesis of DNA which is essential for cell maturation. Vitamin B12 is also concerned in the maintenance of sulphydryl groups in reduced form, deficiency leading to decreased amounts of reduced SH content of erythrocytes and liver cells. Overall, vitamin B12 acts as a coenzyme for various metabolic functions, including fat and carbohydrate metabolism and protein synthesis. It is necessary for growth, cell replication, hematopoiesis, and nucleoprotein as well as myelin synthesis. This is largely due to its effects on metabolism of methionine folic acid, and malonic acid.

Mechanism of action

Vitamin B12 exists in four major forms referred to collectively as cobalamins; deoxyadenosylcobalamin, methylcobalamin, hydroxocobalamin, and cyanocobalamin. Two of these, methylcobalamin and 5-deoxyadenosyl cobalamin, are primarily used by the body. Methionine synthase needs methylcobalamin as a cofactor. This enzyme is involved in the conversion of the amino acid homocysteine into methionine. Methionine in turn is required for DNA methylation. 5-Deoxyadenosyl cobalamin is a cofactor needed by the enzyme that converts L-methylmalonyl-CoA to succinyl-CoA. This conversion is an important step in the extraction of energy from proteins and fats. Furthermore, succinyl CoA is necessary for the production of hemoglobin, the substances that carries oxygen in red blood cells.

TargetActionsOrganism
AMethionine synthase
cofactor
Human
AMethylmalonyl-CoA mutase, mitochondrial
cofactor
Human
UMethionine synthase reductase
other
Human
UMethylmalonic aciduria type A protein, mitochondrial
other/unknown
Human
UTranscobalamin-1
other
Human
UProtein amnionless
other
Human
UCubilin
other
Human
UCob(I)yrinic acid a,c-diamide adenosyltransferase, mitochondrial
other
Human
UMethylmalonic aciduria and homocystinuria type C protein
other/unknown
Human
Absorption

Readily absorbed from the gastrointestinal tract, except in malabsorption syndromes. Vitamin B12 is absorbed in the lower half of the ileum.

Volume of distribution
Not Available
Protein binding

Very high (90%). Cobalamins are extensively bound to two specific plasma proteins called transcobalamin 1 and 2; 70% to transcobalamin 1, 5% to transcobalamin 2.

Metabolism

Primarily hepatic. Cobalamins are absorbed in the ileum and stored in the liver. They continuously undergo enterohepatic recycling via secretion in the bile. Part of a dose is excreted in the urine, most of it in the first 8 hours.

Route of elimination

Each hydroxocobalamin molecule can bind one cyanide ion by substituting it for the hydroxo ligand linked to the trivalent cobalt ion, to form cyanocobalamin, which is then excreted in the urine.

Half life

Approximately 6 days (peak plasma concentration after 8-12 hours from oral administration)

Clearance
Not Available
Toxicity
Not Available
Affected organisms
  • Humans and other mammals
Pathways
Not Available
Pharmacogenomic Effects/ADRs
Not Available

Interactions

Drug Interactions
DrugInteraction
AbacavirHydroxocobalamin may decrease the excretion rate of Abacavir which could result in a higher serum level.
AcarboseHydroxocobalamin may decrease the excretion rate of Acarbose which could result in a higher serum level.
AceclofenacAceclofenac may decrease the excretion rate of Hydroxocobalamin which could result in a higher serum level.
AcemetacinAcemetacin may decrease the excretion rate of Hydroxocobalamin which could result in a higher serum level.
AcetaminophenHydroxocobalamin may decrease the excretion rate of Acetaminophen which could result in a higher serum level.
Acetylsalicylic acidAcetylsalicylic acid may decrease the excretion rate of Hydroxocobalamin which could result in a higher serum level.
AclidiniumHydroxocobalamin may decrease the excretion rate of Aclidinium which could result in a higher serum level.
AcrivastineHydroxocobalamin may decrease the excretion rate of Acrivastine which could result in a higher serum level.
AcyclovirAcyclovir may decrease the excretion rate of Hydroxocobalamin which could result in a higher serum level.
AdefovirAdefovir may decrease the excretion rate of Hydroxocobalamin which could result in a higher serum level.
Food Interactions
Not Available

References

Synthesis Reference

Takayuki Hirayama, Takashi Kiyota, "Process for production of hydroxocobalamin." U.S. Patent US5338418, issued June, 1982.

US5338418
General References
  1. Seetharam B, Yammani RR: Cobalamin transport proteins and their cell-surface receptors. Expert Rev Mol Med. 2003 Jun 13;5(18):1-18. [PubMed:14585166]
External Links
Human Metabolome Database
HMDB0002308
KEGG Drug
D01027
KEGG Compound
C08230
PubChem Compound
70678542
PubChem Substance
46505751
ChemSpider
21403074
ChEBI
27786
ChEMBL
CHEMBL2103737
Therapeutic Targets Database
DAP001336
PharmGKB
PA164768689
RxList
RxList Drug Page
Drugs.com
Drugs.com Drug Page
Wikipedia
Hydroxocobalamin
ATC Codes
B03BA03 — HydroxocobalaminV03AB33 — HydroxocobalaminB03BA53 — Hydroxocobalamin, combinations
AHFS Codes
  • 92:12.00 — Antidotes
  • 88:08.00 — Vitamin B Complex
MSDS
Download (73.6 KB)

Clinical Trials

Clinical Trials
PhaseStatusPurposeConditionsCount
2Not Yet RecruitingTreatmentArterial Hypotension / Cardiac Valve Disease / Coronary Artery Disease / Vasoplegia1
4RecruitingTreatmentVitamin B12 Deficiency1
Not AvailableCompletedBasic ScienceVitamin B 12 Deficiency1

Pharmacoeconomics

Manufacturers
  • Merck and co inc
  • Merck sante sas
  • Abraxis pharmaceutical products
  • Watson laboratories inc
  • Bel mar laboratories inc
Packagers
  • Abraxis BioScience Inc.
  • C.O. Truxton Inc.
  • Dey Pharma LP
  • Letco Medical Inc.
  • Martica Enterprises Inc.
  • Meridian Medical Technologies Inc.
  • Merit Pharmaceuticals
  • Primedics Laboratories
  • Watson Pharmaceuticals
Dosage forms
FormRouteStrength
Injection, powder, for solutionIntravenous2.5 g
Injection, powder, for solutionIntravenous5 g
Injection, powder, lyophilized, for solutionIntravenous2.5 g/100mL
Injection, powder, lyophilized, for solutionIntravenous5 g/250mL
Powder, for solutionIntravenous2.5 g
Powder, for solutionIntravenous5 g
TabletOral
Tablet, chewableOral
LiquidIntramuscular1 mg
Injection, solutionIntramuscular1000 ug/1mL
Prices
Unit descriptionCostUnit
Cyanokit 5 g kit812.5USD kit
Hydroxocobalamin 1000 mcg/ml1.28USD ml
DrugBank does not sell nor buy drugs. Pricing information is supplied for informational purposes only.
Patents
Patent NumberPediatric ExtensionApprovedExpires (estimated)
US5834448No1996-11-142016-11-14Us

Properties

State
Solid
Experimental Properties
Not Available
Predicted Properties
PropertyValueSource
logP-14ChemAxon
pKa (Strongest Acidic)1.82ChemAxon
pKa (Strongest Basic)-0.55ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count14ChemAxon
Hydrogen Donor Count10ChemAxon
Polar Surface Area437.23 Å2ChemAxon
Rotatable Bond Count16ChemAxon
Refractivity340.03 m3·mol-1ChemAxon
Polarizability135.56 Å3ChemAxon
Number of Rings12ChemAxon
Bioavailability0ChemAxon
Rule of FiveNoChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleYesChemAxon
Predicted ADMET features
PropertyValueProbability
Human Intestinal Absorption+0.6922
Blood Brain Barrier-0.712
Caco-2 permeable-0.6447
P-glycoprotein substrateSubstrate0.8579
P-glycoprotein inhibitor INon-inhibitor0.6643
P-glycoprotein inhibitor IIInhibitor0.5
Renal organic cation transporterNon-inhibitor0.9054
CYP450 2C9 substrateNon-substrate0.8081
CYP450 2D6 substrateNon-substrate0.8083
CYP450 3A4 substrateSubstrate0.6458
CYP450 1A2 substrateNon-inhibitor0.8778
CYP450 2C9 inhibitorNon-inhibitor0.8836
CYP450 2D6 inhibitorNon-inhibitor0.8717
CYP450 2C19 inhibitorNon-inhibitor0.709
CYP450 3A4 inhibitorInhibitor0.5841
CYP450 inhibitory promiscuityLow CYP Inhibitory Promiscuity0.8028
Ames testNon AMES toxic0.5675
CarcinogenicityNon-carcinogens0.7831
BiodegradationNot ready biodegradable0.9729
Rat acute toxicity2.6700 LD50, mol/kg Not applicable
hERG inhibition (predictor I)Weak inhibitor0.8966
hERG inhibition (predictor II)Non-inhibitor0.6952
ADMET data is predicted using admetSAR, a free tool for evaluating chemical ADMET properties. (23092397)

Spectra

Mass Spec (NIST)
Not Available
Spectra
SpectrumSpectrum TypeSplash Key
Predicted MS/MS Spectrum - 10V, Positive (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 20V, Positive (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 40V, Positive (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 10V, Negative (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 20V, Negative (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 40V, Negative (Annotated)Predicted LC-MS/MSNot Available

Taxonomy

Description
This compound belongs to the class of organic compounds known as cobalamin derivatives. These are organic compounds containing a corrin ring, a cobalt atom, an a nucleotide moiety. Cobalamin Derivatives are actually derived from vitamin B12.
Kingdom
Organic compounds
Super Class
Organoheterocyclic compounds
Class
Tetrapyrroles and derivatives
Sub Class
Corrinoids
Direct Parent
Cobalamin derivatives
Alternative Parents
Metallotetrapyrroles / Benzimidazole ribonucleosides and ribonucleotides / Pentose phosphates / Glycosylamines / Monosaccharide phosphates / Benzimidazoles / Phosphoethanolamines / Dialkyl phosphates / Fatty amides / Benzenoids
show 19 more
Substituents
Cobalamin / Metallotetrapyrrole skeleton / 1-ribofuranosylbenzimidazole / Pentose phosphate / Glycosyl compound / N-glycosyl compound / Pentose monosaccharide / Monosaccharide phosphate / Benzimidazole / Phosphoethanolamine
show 42 more
Molecular Framework
Aromatic heteropolycyclic compounds
External Descriptors
cobalamins (CHEBI:27786)

Targets

Kind
Protein
Organism
Human
Pharmacological action
Yes
Actions
Cofactor
General Function
Zinc ion binding
Specific Function
Catalyzes the transfer of a methyl group from methyl-cobalamin to homocysteine, yielding enzyme-bound cob(I)alamin and methionine. Subsequently, remethylates the cofactor using methyltetrahydrofola...
Gene Name
MTR
Uniprot ID
Q99707
Uniprot Name
Methionine synthase
Molecular Weight
140525.91 Da
References
  1. Kolhouse JF, Utley C, Stabler SP, Allen RH: Mechanism of conversion of human apo- to holomethionine synthase by various forms of cobalamin. J Biol Chem. 1991 Dec 5;266(34):23010-5. [PubMed:1744096]
  2. Ogier de Baulny H, Gerard M, Saudubray JM, Zittoun J: Remethylation defects: guidelines for clinical diagnosis and treatment. Eur J Pediatr. 1998 Apr;157 Suppl 2:S77-83. [PubMed:9587031]
  3. Tkachuck RD, Weinstein PP, Mueller JF: Metabolic fate of cyanocobalamin taken up by Spirometra mansonoides spargana. J Parasitol. 1977 Aug;63(4):694-700. [PubMed:18565]
  4. Quadros EV, Jacobsen DW: The dynamics of cobalamin utilization in L-1210 mouse leukemia cells: a model of cellular cobalamin metabolism. Biochim Biophys Acta. 1995 Jun 9;1244(2-3):395-403. [PubMed:7599160]
  5. Rosenblatt DS, Thomas IT, Watkins D, Cooper BA, Erbe RW: Vitamin B12 responsive homocystinuria and megaloblastic anemia: heterogeneity in methylcobalamin deficiency. Am J Med Genet. 1987 Feb;26(2):377-83. [PubMed:3812589]
Kind
Protein
Organism
Human
Pharmacological action
Yes
Actions
Cofactor
General Function
Modified amino acid binding
Specific Function
Involved in the degradation of several amino acids, odd-chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species.
Gene Name
MUT
Uniprot ID
P22033
Uniprot Name
Methylmalonyl-CoA mutase, mitochondrial
Molecular Weight
83133.755 Da
References
  1. Willard HF, Rosenberg LE: Inborn errors of cobalamin metabolism: effect of cobalamin supplementation in culture on methylmalonyl CoA mutase activity in normal and mutant human fibroblasts. Biochem Genet. 1979 Feb;17(1-2):57-75. [PubMed:36882]
  2. Dayem LC, Carney JR, Santi DV, Pfeifer BA, Khosla C, Kealey JT: Metabolic engineering of a methylmalonyl-CoA mutase-epimerase pathway for complex polyketide biosynthesis in Escherichia coli. Biochemistry. 2002 Apr 23;41(16):5193-201. [PubMed:11955068]
  3. Mayatepek E, Hoffmann GF, Baumgartner R, Schulze A, Jakobs C, Trefz FK, Bremer HJ: Atypical vitamin B12-unresponsive methylmalonic aciduria in sibship with severe progressive encephalomyelopathy: a new genetic disease? Eur J Pediatr. 1996 May;155(5):398-403. [PubMed:8741039]
  4. Tkachuck RD, Weinstein PP, Mueller JF: Metabolic fate of cyanocobalamin taken up by Spirometra mansonoides spargana. J Parasitol. 1977 Aug;63(4):694-700. [PubMed:18565]
  5. Quadros EV, Jacobsen DW: The dynamics of cobalamin utilization in L-1210 mouse leukemia cells: a model of cellular cobalamin metabolism. Biochim Biophys Acta. 1995 Jun 9;1244(2-3):395-403. [PubMed:7599160]
  6. Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. [PubMed:11752352]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Other
General Function
Oxidoreductase activity, oxidizing metal ions, nad or nadp as acceptor
Specific Function
Involved in the reductive regeneration of cob(I)alamin (vitamin B12) cofactor required for the maintenance of methionine synthase in a functional state. Necessary for utilization of methylgroups fr...
Gene Name
MTRR
Uniprot ID
Q9UBK8
Uniprot Name
Methionine synthase reductase
Molecular Weight
80409.22 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Other/unknown
General Function
Hydrolase activity
Specific Function
Probable GTPase. May function as chaperone. May be involved in the transport of cobalamin (Cbl) into mitochondria for the final steps of adenosylcobalamin (AdoCbl) synthesis.
Gene Name
MMAA
Uniprot ID
Q8IVH4
Uniprot Name
Methylmalonic aciduria type A protein, mitochondrial
Molecular Weight
46537.865 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423]
  3. Manoli I, Sloan JL, Venditti CP: Isolated Methylmalonic Acidemia . [PubMed:20301409]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Other
General Function
Cobalamin binding
Specific Function
Binds vitamin B12 with femtomolar affinity and protects it from the acidic environment of the stomach.
Gene Name
TCN1
Uniprot ID
P20061
Uniprot Name
Transcobalamin-1
Molecular Weight
48206.32 Da
References
  1. MacDonald CM, Farquharson J, Bessent RG, Adams JF: The forms of vitamin B12 on the transcobalamins. Clin Sci Mol Med. 1977 Feb;52(2):215-8. [PubMed:844253]
  2. Rothenberg SP, Marcoullis GP, Schwarz S, Lader E: Measurement of cyanocobalamin in serum by a specific radioimmunoassay. J Lab Clin Med. 1984 Jun;103(6):959-72. [PubMed:6726060]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Other
General Function
Receptor binding
Specific Function
Necessary for efficient absorption of vitamin B12. May direct the production of trunk mesoderm during development by modulating a bone morphogenetic protein (BMP) signaling pathway in the underlyin...
Gene Name
AMN
Uniprot ID
Q9BXJ7
Uniprot Name
Protein amnionless
Molecular Weight
47753.91 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423]
  3. Seetharam B, Yammani RR: Cobalamin transport proteins and their cell-surface receptors. Expert Rev Mol Med. 2003 Jun 13;5(18):1-18. [PubMed:14585166]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Other
General Function
Transporter activity
Specific Function
Cotransporter which plays a role in lipoprotein, vitamin and iron metabolism, by facilitating their uptake. Binds to ALB, MB, Kappa and lambda-light chains, TF, hemoglobin, GC, SCGB1A1, APOA1, high...
Gene Name
CUBN
Uniprot ID
O60494
Uniprot Name
Cubilin
Molecular Weight
398732.93 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423]
  3. Seetharam B, Yammani RR: Cobalamin transport proteins and their cell-surface receptors. Expert Rev Mol Med. 2003 Jun 13;5(18):1-18. [PubMed:14585166]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Other
General Function
Cob(i)yrinic acid a,c-diamide adenosyltransferase activity
Specific Function
Not Available
Gene Name
MMAB
Uniprot ID
Q96EY8
Uniprot Name
Cob(I)yrinic acid a,c-diamide adenosyltransferase, mitochondrial
Molecular Weight
27387.975 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Other/unknown
General Function
Cobalamin binding
Specific Function
May be involved in the binding and intracellular trafficking of cobalamin (vitamin B12).
Gene Name
MMACHC
Uniprot ID
Q9Y4U1
Uniprot Name
Methylmalonic aciduria and homocystinuria type C protein
Molecular Weight
31728.095 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423]
  3. Lerner-Ellis JP, Tirone JC, Pawelek PD, Dore C, Atkinson JL, Watkins D, Morel CF, Fujiwara TM, Moras E, Hosack AR, Dunbar GV, Antonicka H, Forgetta V, Dobson CM, Leclerc D, Gravel RA, Shoubridge EA, Coulton JW, Lepage P, Rommens JM, Morgan K, Rosenblatt DS: Identification of the gene responsible for methylmalonic aciduria and homocystinuria, cblC type. Nat Genet. 2006 Jan;38(1):93-100. Epub 2005 Nov 27. [PubMed:16311595]

Carriers

Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Substrate
General Function
Metal ion binding
Specific Function
Primary vitamin B12-binding and transport protein. Delivers cobalamin to cells.
Gene Name
TCN2
Uniprot ID
P20062
Uniprot Name
Transcobalamin-2
Molecular Weight
47534.54 Da
References
  1. Begley JA, Colligan PD, Chu RC: Transcobalamin II mediated delivery of albumin-bound hydroxocobalamin to human liver cells. Proc Soc Exp Biol Med. 1993 Nov;204(2):206-10. [PubMed:8415778]
  2. Skouby AP, Hippe E, Olesen H: Antibody to transcobalamin II and B12 binding capacity in patients treated with hydroxocobalamin. Blood. 1971 Dec;38(6):769-74. [PubMed:5126165]
  3. Hippe E, Olesen H, Skouby A: [Occurrence of antibodies against transcobalamin II in relation to the number of and the intervals between depot therapy with hydroxocobalamin]. Nord Med. 1970 Dec 3;84(49):1570. [PubMed:5488577]
  4. Brown KL, Marques HM, Jacobsen DW: Heteronuclear NMR studies of cobalamins. 31P NMR observations of cobalamins bound to a haptocorrin from chicken serum. J Biol Chem. 1988 Feb 5;263(4):1872-7. [PubMed:3338997]
  5. Chu RC, Begley JA, Colligan PD, Hall CA: The methylcobalamin metabolism of cultured human fibroblasts. Metabolism. 1993 Mar;42(3):315-9. [PubMed:8487649]
  6. Seetharam B, Yammani RR: Cobalamin transport proteins and their cell-surface receptors. Expert Rev Mol Med. 2003 Jun 13;5(18):1-18. [PubMed:14585166]

Drug created on June 13, 2005 07:24 / Updated on November 14, 2018 12:39