3-methylbutyrylcarnitine profile

3-methylbutyrylcarnitine: affects 2-oxo acid dehydrogenase activity in intact mitochondria of rat muscle; RN given refers to (R)-isomer [Medical Subject Headings (MeSH), National Library of Medicine, extracted Dec-2023]

O-isovalerylcarnitine : A C5-acylcarnitine having isovaleryl as the acyl substituent. [Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res]

ID Source	ID
PubMed CID	6426851
CHEBI ID	73025
SCHEMBL ID	232682
MeSH ID	M0088943

Synonym
AKOS015841719
3-methylbutyrylcarnitine
31023-24-2
3-(3-methylbutanoyloxy)-4-(trimethylazaniumyl)butanoate
isovalerylcarnitine
SCHEMBL232682
CHEBI:73025
3-[(3-methylbutanoyl)oxy]-4-(trimethylammonio)butanoate
o-isovalerylcarnitine
isovaleroyl carnitine
IGQBPDJNUXPEMT-UHFFFAOYSA-N
1-propanaminium, 3-carboxy-n,n,n-trimethyl-2-(3-methyl-1-oxobutoxy)-, inner salt, (2r)-
LMFA07070076
3-[(3-methylbutanoyl)oxy]-4-(trimethylazaniumyl)butanoate
isovaleryl l-carnitine
Q27140242

Role	Description
human metabolite	Any mammalian metabolite produced during a metabolic reaction in humans (Homo sapiens).
[role information is derived from Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res]

Class	Description
C5-acylcarnitine	Any O-acylcarnitine in which the acyl group contains a total of 5 carbon atoms.
[compound class information is derived from Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res]

Pathway	Proteins	Compounds
Leucine, isoleucine and valine metabolism	24	70

Timeframe	Studies, This Drug (%)	All Drugs %
pre-1990	8 (27.59)	18.7374
1990's	11 (37.93)	18.2507
2000's	4 (13.79)	29.6817
2010's	5 (17.24)	24.3611
2020's	1 (3.45)	2.80
[information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023]

Publication Type	This drug (%)	All Drugs (%)
Trials	0 (0.00%)	5.53%
Reviews	2 (6.90%)	6.00%
Case Studies	5 (17.24%)	4.05%
Observational	0 (0.00%)	0.25%
Other	22 (75.86%)	84.16%
[information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023]

Substance	Relationship Strength	Studies	Classes	Roles
acetylcarnitine Acetylcarnitine: An acetic acid ester of CARNITINE that facilitates movement of ACETYL COA into the matrices of mammalian MITOCHONDRIA during the oxidation of FATTY ACIDS.	4.01	4	O-acylcarnitine	human metabolite
2-keto-4-methylvalerate alpha-ketoisocaproic acid: RN given refers to parent cpd. 4-methyl-2-oxopentanoate : A 2-oxo monocarboxylic acid anion that is the conjugate base of 4-methyl-2-oxopentanoic acid.. 4-methyl-2-oxopentanoic acid : A 2-oxo monocarboxylic acid that is pentanoic acid (valeric acid) substituted with a keto group at C-2 and a methyl group at C-4. A metabolite that has been found to accumulate in maple syrup urine disease.	1.97	1	2-oxo monocarboxylic acid; branched-chain keto acid	algal metabolite; human metabolite
carnitine [no description available]	7.22	29	amino-acid betaine	human metabolite; mouse metabolite
methylmalonic acid Methylmalonic Acid: A malonic acid derivative which is a vital intermediate in the metabolism of fat and protein. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This metabolic disease is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA.. methylmalonic acid : A dicarboxylic acid that is malonic acid in which one of the methylene hydrogens is substituted by a methyl group.	1.96	1	C4-dicarboxylic acid	human metabolite
glutaric acid glutaric acid: RN given refers to parent cpd. glutaric acid : An alpha,omega-dicarboxylic acid that is a linear five-carbon dicarboxylic acid.	1.97	1	alpha,omega-dicarboxylic acid; dicarboxylic fatty acid	Daphnia magna metabolite; human metabolite
glycine [no description available]	4.3	7	alpha-amino acid; amino acid zwitterion; proteinogenic amino acid; serine family amino acid	EC 2.1.2.1 (glycine hydroxymethyltransferase) inhibitor; fundamental metabolite; hepatoprotective agent; micronutrient; neurotransmitter; NMDA receptor agonist; nutraceutical
alanine Alanine: A non-essential amino acid that occurs in high levels in its free state in plasma. It is produced from pyruvate by transamination. It is involved in sugar and acid metabolism, increases IMMUNITY, and provides energy for muscle tissue, BRAIN, and the CENTRAL NERVOUS SYSTEM.. alanine : An alpha-amino acid that consists of propionic acid bearing an amino substituent at position 2.	1.98	1	alanine zwitterion; alanine; L-alpha-amino acid; proteinogenic amino acid; pyruvate family amino acid	EC 4.3.1.15 (diaminopropionate ammonia-lyase) inhibitor; fundamental metabolite
glutamine Glutamine: A non-essential amino acid present abundantly throughout the body and is involved in many metabolic processes. It is synthesized from GLUTAMIC ACID and AMMONIA. It is the principal carrier of NITROGEN in the body and is an important energy source for many cells.. L-glutamine : An optically active form of glutamine having L-configuration.. glutamine : An alpha-amino acid that consists of butyric acid bearing an amino substituent at position 2 and a carbamoyl substituent at position 4.	2.42	2	amino acid zwitterion; glutamine family amino acid; glutamine; L-alpha-amino acid; polar amino acid zwitterion; proteinogenic amino acid	EC 1.14.13.39 (nitric oxide synthase) inhibitor; Escherichia coli metabolite; human metabolite; metabolite; micronutrient; mouse metabolite; nutraceutical; Saccharomyces cerevisiae metabolite
lysine Lysine: An essential amino acid. It is often added to animal feed.. lysine : A diamino acid that is caproic (hexanoic) acid bearing two amino substituents at positions 2 and 6.. L-lysine : An L-alpha-amino acid; the L-isomer of lysine.	1.96	1	aspartate family amino acid; L-alpha-amino acid zwitterion; L-alpha-amino acid; lysine; organic molecular entity; proteinogenic amino acid	algal metabolite; anticonvulsant; Escherichia coli metabolite; human metabolite; micronutrient; mouse metabolite; nutraceutical; plant metabolite; Saccharomyces cerevisiae metabolite
leucine Leucine: An essential branched-chain amino acid important for hemoglobin formation.. leucine : A branched-chain amino acid that consists of glycine in which one of the hydrogens attached to the alpha-carbon is substituted by an isobutyl group.	3.99	5	amino acid zwitterion; L-alpha-amino acid; leucine; proteinogenic amino acid; pyruvate family amino acid	algal metabolite; Escherichia coli metabolite; human metabolite; mouse metabolite; plant metabolite; Saccharomyces cerevisiae metabolite
valine Valine: A branched-chain essential amino acid that has stimulant activity. It promotes muscle growth and tissue repair. It is a precursor in the penicillin biosynthetic pathway.. valine : A branched-chain amino acid that consists of glycine in which one of the hydrogens attached to the alpha-carbon is substituted by an isopropyl group.. L-valine : The L-enantiomer of valine.	1.99	1	L-alpha-amino acid zwitterion; L-alpha-amino acid; proteinogenic amino acid; pyruvate family amino acid; valine	algal metabolite; Escherichia coli metabolite; human metabolite; micronutrient; mouse metabolite; nutraceutical; Saccharomyces cerevisiae metabolite
isoleucine Isoleucine: An essential branched-chain aliphatic amino acid found in many proteins. It is an isomer of LEUCINE. It is important in hemoglobin synthesis and regulation of blood sugar and energy levels.. isoleucine : A 2-amino-3-methylpentanoic acid having either (2R,3R)- or (2S,3S)-configuration.. L-isoleucine : The L-enantiomer of isoleucine.	3.11	1	aspartate family amino acid; isoleucine; L-alpha-amino acid zwitterion; L-alpha-amino acid; proteinogenic amino acid	algal metabolite; Escherichia coli metabolite; human metabolite; mouse metabolite; plant metabolite; Saccharomyces cerevisiae metabolite
isovaleric acid isovaleric acid: structure. isovaleric acid : A C5, branched-chain saturated fatty acid.	4.29	7	branched-chain saturated fatty acid; methylbutyric acid; short-chain fatty acid	mammalian metabolite; plant metabolite
2-cyclohexen-1-one 2-cyclohexen-1-one: RN given refers to unlabeled cpd with specified locant for double bond. cyclohexenone : The parent compound of the cyclohexenones, composed of cyclohexanone having one double bond in the ring.. cyclohex-2-enone : A cyclohexenone having its C=C double bond at the 2-position.	1.98	1	cyclohexenone
tiletamine hydrochloride Cyclohexanones: Cyclohexane ring substituted by one or more ketones in any position.. cyclohexanones : Any alicyclic ketone based on a cyclohexane skeleton and its substituted derivatives thereof.	1.98	1
glutamic acid Glutamic Acid: A non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.. glutamic acid : An alpha-amino acid that is glutaric acid bearing a single amino substituent at position 2.	2.08	1	glutamic acid; glutamine family amino acid; L-alpha-amino acid; proteinogenic amino acid	Escherichia coli metabolite; ferroptosis inducer; micronutrient; mouse metabolite; neurotransmitter; nutraceutical
s-adenosylmethionine acylcarnitine: structure in first source. S-adenosyl-L-methioninate : A sulfonium betaine that is a conjugate base of S-adenosyl-L-methionine obtained by the deprotonation of the carboxy group.	4.06	4	sulfonium betaine	human metabolite
beta-hydroxyisovaleric acid 3-hydroxyisovaleric acid : A 3-hydroxy monocarboxylic acid that is isovaleric acid substituted at position 3 by a hydroxy group. Used as indicator of biotin deficiency.	1.97	1	3-hydroxy monocarboxylic acid	human metabolite
propionylcarnitine propionylcarnitine: RN given refers to cpd without isomeric designation	3.79	3	O-acylcarnitine	analgesic; antirheumatic drug; cardiotonic drug; human metabolite; peripheral nervous system drug
valerates Valerates: Derivatives of valeric acid, including its salts and esters.	2.37	2	short-chain fatty acid anion; straight-chain saturated fatty acid anion	plant metabolite
4'-bromophenacyl triflate [no description available]	1.97	1
sulbactam [no description available]	1.99	1	penicillanic acids
isobutyryl-1-carnitine O-isobutyryl-L-carnitine : An optically active form of O-isobutyrylcarnitine having (R)-configuration.	2.67	3	methyl-branched fatty acyl-L-carnitine; O-isobutyrylcarnitine; saturated fatty acyl-L-carnitine	human metabolite
butyrylcarnitine butyrylcarnitine: structure in first source. O-butanoyl-L-carnitine : An optically active form of O-butanoylcarnitine having L-configuration.. O-butanoylcarnitine : A C4-acylcarnitine that is the O-butanoyl derivative of carnitine.	2.08	1	O-butanoylcarnitine; saturated fatty acyl-L-carnitine	metabolite
n-isovalerylglycine N-isovalerylglycine: urinary metabolite of isovaleric acid in isovaleric acidemia (sweaty feet syndrome). N-isovalerylglycine : An N-acylglycine in which the acyl group is specified as isovaleryl.	3.83	4	N-acylglycine	human urinary metabolite
hexanoylcarnitine hexanoylcarnitine: RN given refers to chloride salt; RN for parent cpd not available 11/89. O-hexanoylcarnitine : An O-acylcarnitine compound having hexanoyl as the acyl substituent.	2.08	1	hexanoate ester; O-acylcarnitine	human metabolite
decanoylcarnitine decanoylcarnitine: therapeutic agent for diabetic acidosis; RN given refers to cpd without isomeric designation. O-decanoylcarnitine : An O-acylcarnitine compound having decanoyl as the acyl substituent.	2.06	1	decanoate ester; O-acylcarnitine	human urinary metabolite
octanoylcarnitine octanoylcarnitine: RN given refers to cpd without isomeric designation. O-octanoyl-L-carnitine : The L-enantiomer of an O-octanoylcarnitine.	2.4	2	O-octanoylcarnitine; saturated fatty acyl-L-carnitine	human metabolite
calpain Calpain: Cysteine proteinase found in many tissues. Hydrolyzes a variety of endogenous proteins including NEUROPEPTIDES; CYTOSKELETAL PROTEINS; proteins from SMOOTH MUSCLE; CARDIAC MUSCLE; liver; platelets; and erythrocytes. Two subclasses having high and low calcium sensitivity are known. Removes Z-discs and M-lines from myofibrils. Activates phosphorylase kinase and cyclic nucleotide-independent protein kinase. This enzyme was formerly listed as EC 3.4.22.4.	3.07	5
glutarylcarnitine glutarylcarnitine: found in glutaric aciduria type 1	1.97	1	O-acylcarnitine
calpastatin calpastatin: large mol wt, heat-stable calpain inhibitor; not based on sequestering Ca++ from medium, but binding to calpain does require Ca++. calpastatin peptide Ac 184-210 : A 27-membered polypeptide comprising the sequence Ac-Asp-Pro-Met-Ser-Ser-Thr-Tyr-Ile-Glu-Glu-Leu-Gly-Lys-Arg-Glu-Val-Thr-Ile-Pro-Pro-Lys-Tyr-Arg-Glu-Leu-Leu-Ala-NH2. An acetylated synthetic peptide from human calpastatin that strongly inhibits both calpains I and II but not papain (a cysteine protease) or trypsin (a serine protease).	1.98	1	polypeptide	EC 3.4.22.52 (calpain-1) inhibitor; EC 3.4.22.53 (calpain-2) inhibitor
pivaloylcarnitine O-pivaloylcarnitine : A C5-acylcarnitine in which the acyl group specified is pivaloyl.	2.43	2	C5-acylcarnitine	metabolite

Condition	Relationship Strength	Studies
Cancer of Lung [description not available]	2.41	1
Lung Neoplasms Tumors or cancer of the LUNG.	2.41	1
Amino Acid Metabolism Disorders, Inborn [description not available]	5.44	9
Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH.	2.9	4
Asymptomatic Conditions [description not available]	2.15	1
Chronic Illness [description not available]	2.15	1
Acute Disease Disease having a short and relatively severe course.	2.15	1
Chronic Disease Diseases which have one or more of the following characteristics: they are permanent, leave residual disability, are caused by nonreversible pathological alteration, require special training of the patient for rehabilitation, or may be expected to require a long period of supervision, observation, or care (Dictionary of Health Services Management, 2d ed). For epidemiological studies chronic disease often includes HEART DISEASES; STROKE; CANCER; and diabetes (DIABETES MELLITUS, TYPE 2).	2.15	1
Cardiomyopathy, Congestive [description not available]	2.06	1
Malabsorption Syndromes General term for a group of MALNUTRITION syndromes caused by failure of normal INTESTINAL ABSORPTION of nutrients.	2.06	1
Bowel Diseases, Inflammatory [description not available]	2.06	1
Cardiomyopathy, Dilated A form of CARDIAC MUSCLE disease that is characterized by ventricular dilation, VENTRICULAR DYSFUNCTION, and HEART FAILURE. Risk factors include SMOKING; ALCOHOL DRINKING; HYPERTENSION; INFECTION; PREGNANCY; and mutations in the LMNA gene encoding LAMIN TYPE A, a NUCLEAR LAMINA protein.	2.06	1
Inflammatory Bowel Diseases Chronic, non-specific inflammation of the GASTROINTESTINAL TRACT. Etiology may be genetic or environmental. This term includes CROHN DISEASE and ULCERATIVE COLITIS.	2.06	1
Cognition Disorders Disorders characterized by disturbances in mental processes related to learning, thinking, reasoning, and judgment.	2.99	1
Cirrhosis, Liver [description not available]	2.99	1
Nervous System Disorders [description not available]	2.99	1
Cardiovascular Diseases Pathological conditions involving the CARDIOVASCULAR SYSTEM including the HEART; the BLOOD VESSELS; or the PERICARDIUM.	2.99	1
Depression Depressive states usually of moderate intensity in contrast with MAJOR DEPRESSIVE DISORDER present in neurotic and psychotic disorders.	2.99	1
Diabetes Mellitus A heterogeneous group of disorders characterized by HYPERGLYCEMIA and GLUCOSE INTOLERANCE.	2.99	1
Liver Cirrhosis Liver disease in which the normal microcirculation, the gross vascular anatomy, and the hepatic architecture have been variably destroyed and altered with fibrous septa surrounding regenerated or regenerating parenchymal nodules.	2.99	1
Nervous System Diseases Diseases of the central and peripheral nervous system. This includes disorders of the brain, spinal cord, cranial nerves, peripheral nerves, nerve roots, autonomic nervous system, neuromuscular junction, and muscle.	2.99	1
Deficiency, Mental [description not available]	2.93	1
Intellectual Disability Subnormal intellectual functioning which originates during the developmental period. This has multiple potential etiologies, including genetic defects and perinatal insults. Intelligence quotient (IQ) scores are commonly used to determine whether an individual has an intellectual disability. IQ scores between 70 and 79 are in the borderline range. Scores below 67 are in the disabled range. (from Joynt, Clinical Neurology, 1992, Ch55, p28)	2.93	1
Colitis Gravis [description not available]	2.03	1
Colitis, Ulcerative Inflammation of the COLON that is predominantly confined to the MUCOSA. Its major symptoms include DIARRHEA, rectal BLEEDING, the passage of MUCUS, and ABDOMINAL PAIN.	2.03	1
Blood Pressure, High [description not available]	1.98	1
Hypertension Persistently high systemic arterial BLOOD PRESSURE. Based on multiple readings (BLOOD PRESSURE DETERMINATION), hypertension is currently defined as when SYSTOLIC PRESSURE is consistently greater than 140 mm Hg or when DIASTOLIC PRESSURE is consistently 90 mm Hg or more.	1.98	1
Inborn Errors of Metabolism [description not available]	1.99	1
Metabolism, Inborn Errors Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero.	1.99	1
Urinary Tract Infections Inflammatory responses of the epithelium of the URINARY TRACT to microbial invasions. They are often bacterial infections with associated BACTERIURIA and PYURIA.	1.99	1
Cardiomyopathies, Primary [description not available]	1.99	1
Cardiomyopathies A group of diseases in which the dominant feature is the involvement of the CARDIAC MUSCLE itself. Cardiomyopathies are classified according to their predominant pathophysiological features (DILATED CARDIOMYOPATHY; HYPERTROPHIC CARDIOMYOPATHY; RESTRICTIVE CARDIOMYOPATHY) or their etiological/pathological factors (CARDIOMYOPATHY, ALCOHOLIC; ENDOCARDIAL FIBROELASTOSIS).	1.99	1
Aging The gradual irreversible changes in structure and function of an organism that occur as a result of the passage of time.	1.98	1
Embryopathies [description not available]	1.97	1

3-methylbutyrylcarnitine

Description

Cross-References

Synonyms (16)

Roles (1)

Drug Classes (1)

Pathways (1)

Research

Studies (29)

Market Indicators

Research Demand Index: 11.19

Study Types