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Details

Stereochemistry ACHIRAL
Molecular Formula C6H12O2
Molecular Weight 116.1583
Optical Activity NONE
Defined Stereocenters 0 / 0
E/Z Centers 0
Charge 0

SHOW SMILES / InChI
Structure of Dimebutic Acid

SMILES

CCC(C)(C)C(O)=O

InChI

InChIKey=VUAXHMVRKOTJKP-UHFFFAOYSA-N
InChI=1S/C6H12O2/c1-4-6(2,3)5(7)8/h4H2,1-3H3,(H,7,8)

HIDE SMILES / InChI

Molecular Formula C6H12O2
Molecular Weight 116.1583
Charge 0
Count
Stereochemistry ACHIRAL
Additional Stereochemistry No
Defined Stereocenters 0 / 0
E/Z Centers 0
Optical Activity NONE

2,2-DIMETHYLBUTYRIC ACID (HQK-1001) is an orally administered SCFAD (Short Chain Fatty Acid Derivative), which has shown an excellent safety profile and biologic effects on fetal hemoglobin induction and red blood cell production in the laboratory, relevant animal models, and in clinical trials carried out in healthy human subjects as well as patients with sickle cell disease and beta thalassemia. The compound has received Orphan Drug Designation in the United States and Europe for both sickle cell disease and beta thalassemia. HemaQuest Pharmaceuticals was developing HQK-1001 for the oral treatment of sickle cell anaemia and beta thalassaemia. HQK-1001 has been evaluated in phase II trials for beta thalassaemia and sickle cell anaemia.

Approval Year

PubMed

PubMed

TitleDatePubMed
[Chemical components of essential oils from the herb of Ligularia virgaurea].
2003 Jul
New polynuclear manganese clusters from the use of the hydrophobic carboxylate ligand 2,2-dimethylbutyrate.
2005 Jun 27
Top-down systems biology integration of conditional prebiotic modulated transgenomic interactions in a humanized microbiome mouse model.
2008
Fetal globin gene inducers: novel agents and new potential.
2010 Aug
Biotechnological production and applications of statins.
2010 Jan
Patents

Sample Use Guides

Hemoglobin SS or S/β(0) thalassemia: HQK-1001 was administered daily for 26 weeks at 30 mg/kg (n = 15), 40 mg/kg (n = 18) and 50 mg/kg (n = 19), either alone (n = 21) or with hydroxyurea (n = 31).
Route of Administration: Oral
Substance Class Chemical
Created
by admin
on Thu Jul 06 08:23:41 UTC 2023
Edited
by admin
on Thu Jul 06 08:23:41 UTC 2023
Record UNII
AY606CN05O
Record Status Validated (UNII)
Record Version
  • Download
Name Type Language
Dimebutic Acid
INN  
Official Name English
NSC-16045
Code English
NSC-741804
Code English
2,2-DIMETHYLBUTANOIC ACID
Systematic Name English
Butanoic acid, 2,2-dimethyl-
Systematic Name English
Dimebutic acid [INN]
Common Name English
2,2-DIMETHYLBUTYRIC ACID
Systematic Name English
Classification Tree Code System Code
FDA ORPHAN DRUG 746120
Created by admin on Thu Jul 06 08:23:41 UTC 2023 , Edited by admin on Thu Jul 06 08:23:41 UTC 2023
FDA ORPHAN DRUG 751020
Created by admin on Thu Jul 06 08:23:41 UTC 2023 , Edited by admin on Thu Jul 06 08:23:41 UTC 2023
Code System Code Type Description
CAS
595-37-9
Created by admin on Thu Jul 06 08:23:41 UTC 2023 , Edited by admin on Thu Jul 06 08:23:41 UTC 2023
PRIMARY
NSC
741804
Created by admin on Thu Jul 06 08:23:41 UTC 2023 , Edited by admin on Thu Jul 06 08:23:41 UTC 2023
PRIMARY
NCI_THESAURUS
C190420
Created by admin on Thu Jul 06 08:23:41 UTC 2023 , Edited by admin on Thu Jul 06 08:23:41 UTC 2023
PRIMARY
ECHA (EC/EINECS)
209-865-0
Created by admin on Thu Jul 06 08:23:41 UTC 2023 , Edited by admin on Thu Jul 06 08:23:41 UTC 2023
PRIMARY
FDA UNII
AY606CN05O
Created by admin on Thu Jul 06 08:23:41 UTC 2023 , Edited by admin on Thu Jul 06 08:23:41 UTC 2023
PRIMARY
CHEBI
38649
Created by admin on Thu Jul 06 08:23:41 UTC 2023 , Edited by admin on Thu Jul 06 08:23:41 UTC 2023
PRIMARY
NSC
16045
Created by admin on Thu Jul 06 08:23:41 UTC 2023 , Edited by admin on Thu Jul 06 08:23:41 UTC 2023
PRIMARY
EPA CompTox
DTXSID0032811
Created by admin on Thu Jul 06 08:23:41 UTC 2023 , Edited by admin on Thu Jul 06 08:23:41 UTC 2023
PRIMARY
INN
12144
Created by admin on Thu Jul 06 08:23:41 UTC 2023 , Edited by admin on Thu Jul 06 08:23:41 UTC 2023
PRIMARY
SMS_ID
100000126089
Created by admin on Thu Jul 06 08:23:41 UTC 2023 , Edited by admin on Thu Jul 06 08:23:41 UTC 2023
PRIMARY
EVMPD
SUB33307
Created by admin on Thu Jul 06 08:23:41 UTC 2023 , Edited by admin on Thu Jul 06 08:23:41 UTC 2023
PRIMARY
PUBCHEM
11684
Created by admin on Thu Jul 06 08:23:41 UTC 2023 , Edited by admin on Thu Jul 06 08:23:41 UTC 2023
PRIMARY
Related Record Type Details
SALT/SOLVATE -> PARENT
Related Record Type Details
PARENT -> METABOLITE
PLASMA
Related Record Type Details
ACTIVE MOIETY
treatment of propionic acidemia (PA) and methylmalonic acidemia (MMA)