glutarylcarnitine: found in glutaric aciduria type 1 [Medical Subject Headings (MeSH), National Library of Medicine, extracted Dec-2023]
| ID Source | ID |
|---|---|
| PubMed CID | 53481622 |
| CHEBI ID | 165628 |
| MeSH ID | M0150677 |
| Synonym |
|---|
| glutarylcarnitine |
| car(dc5:0) |
| (3s)-3-(4-carboxybutanoyloxy)-4-(trimethylazaniumyl)butanoate |
| CHEBI:165628 |
| (3s)-3-[(4-carboxybutanoyl)oxy]-4-(trimethylazaniumyl)butanoate |
| LMFA07070066 |
| Excerpt | Reference | Relevance |
|---|---|---|
| " P6C forms a complex with pyridoxal phosphate (PLP), a key vitamer of pyridoxine, thereby reducing PLP bioavailability and subsequently causing epilepsy." | ( Inherited Disorders of Lysine Metabolism: A Review. Bouchereau, J; Schiff, M, 2020) | 0.56 |
| Class | Description |
|---|---|
| O-acylcarnitine | Any carboxylic ester obtained by the O-acylation of carnitine. |
| [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] | |
| Timeframe | Studies, This Drug (%) | All Drugs % |
|---|---|---|
| pre-1990 | 2 (7.69) | 18.7374 |
| 1990's | 2 (7.69) | 18.2507 |
| 2000's | 9 (34.62) | 29.6817 |
| 2010's | 9 (34.62) | 24.3611 |
| 2020's | 4 (15.38) | 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 (7.41%) | 6.00% |
| Case Studies | 8 (29.63%) | 4.05% |
| Observational | 0 (0.00%) | 0.25% |
| Other | 17 (62.96%) | 84.16% |
| [information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023] | ||
| Substance | Relationship Strength | Studies | Trials | 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. | 7.37 | 2 | 0 | O-acylcarnitine | human metabolite |
| 1-butanol 1-Butanol: A four carbon linear hydrocarbon that has a hydroxy group at position 1.. butan-1-ol : A primary alcohol that is butane in which a hydrogen of one of the methyl groups is substituted by a hydroxy group. It it produced in small amounts in humans by the gut microbes. | 2.05 | 1 | 0 | alkyl alcohol; primary alcohol; short-chain primary fatty alcohol | human metabolite; mouse metabolite; protic solvent |
| carnitine [no description available] | 5.74 | 26 | 0 | amino-acid betaine | human metabolite; mouse metabolite |
| 2-aminoadipic acid 2-Aminoadipic Acid: A metabolite in the principal biochemical pathway of lysine. It antagonizes neuroexcitatory activity modulated by the glutamate receptor, N-METHYL-D-ASPARTATE; (NMDA).. 2-aminoadipic acid : An alpha-amino acid that is adipic acid bearing a single amino substituent at position 2. An intermediate in the formation of lysine. | 3.62 | 2 | 0 | amino dicarboxylic acid; dicarboxylic fatty acid; non-proteinogenic alpha-amino acid | Caenorhabditis elegans metabolite; mammalian 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. | 5 | 12 | 0 | alpha,omega-dicarboxylic acid; dicarboxylic fatty acid | Daphnia magna metabolite; human metabolite |
| pyridoxal phosphate Pyridoxal Phosphate: This is the active form of VITAMIN B 6 serving as a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. During transamination of amino acids, pyridoxal phosphate is transiently converted into pyridoxamine phosphate (PYRIDOXAMINE).. pyridoxal 5'-phosphate : The monophosphate ester obtained by condensation of phosphoric acid with the primary hydroxy group of pyridoxal. | 3.35 | 1 | 0 | methylpyridines; monohydroxypyridine; pyridinecarbaldehyde; vitamin B6 phosphate | coenzyme; cofactor; EC 2.7.7.7 (DNA-directed DNA polymerase) inhibitor; Escherichia coli metabolite; human metabolite; mouse metabolite; Saccharomyces cerevisiae metabolite |
| pyridoxine 4,5-bis(hydroxymethyl)-2-methylpyridin-3-ol: structure in first source. vitamin B6 : Any member of the group of pyridines that exhibit biological activity against vitamin B6 deficiency. Vitamin B6 deficiency is associated with microcytic anemia, electroencephalographic abnormalities, dermatitis with cheilosis (scaling on the lips and cracks at the corners of the mouth) and glossitis (swollen tongue), depression and confusion, and weakened immune function. Vitamin B6 consists of the vitamers pyridoxine, pyridoxal, and pyridoxamine and their respective 5'-phosphate esters (and includes their corresponding ionized and salt forms). | 3.35 | 1 | 0 | hydroxymethylpyridine; methylpyridines; monohydroxypyridine; vitamin B6 | cofactor; Escherichia coli metabolite; human metabolite; mouse metabolite; Saccharomyces cerevisiae metabolite |
| vigabatrin [no description available] | 2.01 | 1 | 0 | gamma-amino acid | anticonvulsant; EC 2.6.1.19 (4-aminobutyrate--2-oxoglutarate transaminase) inhibitor |
| 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. | 4.18 | 4 | 0 | 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. | 2.05 | 1 | 0 | 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 |
| methionine Methionine: A sulfur-containing essential L-amino acid that is important in many body functions.. methionine : A sulfur-containing amino acid that is butyric acid bearing an amino substituent at position 2 and a methylthio substituent at position 4. | 2.05 | 1 | 0 | aspartate family amino acid; L-alpha-amino acid; methionine zwitterion; methionine; proteinogenic amino acid | antidote to paracetamol poisoning; human metabolite; micronutrient; mouse metabolite; nutraceutical |
| phenylalanine Phenylalanine: An essential aromatic amino acid that is a precursor of MELANIN; DOPAMINE; noradrenalin (NOREPINEPHRINE), and THYROXINE.. L-phenylalanine : The L-enantiomer of phenylalanine.. phenylalanine : An aromatic amino acid that is alanine in which one of the methyl hydrogens is substituted by a phenyl group. | 2.05 | 1 | 0 | amino acid zwitterion; erythrose 4-phosphate/phosphoenolpyruvate family amino acid; L-alpha-amino acid; phenylalanine; proteinogenic amino acid | algal metabolite; EC 3.1.3.1 (alkaline phosphatase) inhibitor; Escherichia coli metabolite; human xenobiotic metabolite; micronutrient; mouse metabolite; nutraceutical; plant metabolite; Saccharomyces cerevisiae metabolite |
| arginine Arginine: An essential amino acid that is physiologically active in the L-form.. arginine : An alpha-amino acid that is glycine in which the alpha-is substituted by a 3-guanidinopropyl group. | 3.62 | 2 | 0 | arginine; glutamine family amino acid; L-alpha-amino acid; proteinogenic amino acid | biomarker; Escherichia coli metabolite; micronutrient; mouse metabolite; nutraceutical |
| n-methylaspartate N-Methylaspartate: An amino acid that, as the D-isomer, is the defining agonist for the NMDA receptor subtype of glutamate receptors (RECEPTORS, NMDA).. N-methyl-D-aspartic acid : An aspartic acid derivative having an N-methyl substituent and D-configuration. | 2.01 | 1 | 0 | amino dicarboxylic acid; D-alpha-amino acid; D-aspartic acid derivative; secondary amino compound | neurotransmitter agent |
| 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. | 3.3 | 6 | 0 | sulfonium betaine | human metabolite |
| coenzyme a [no description available] | 2.41 | 1 | 0 | adenosine 3',5'-bisphosphate | coenzyme; Escherichia coli metabolite; mouse metabolite |
| propionylcarnitine propionylcarnitine: RN given refers to cpd without isomeric designation | 7.4 | 2 | 0 | O-acylcarnitine | analgesic; antirheumatic drug; cardiotonic drug; human metabolite; peripheral nervous system drug |
| allysine L-allysine : An optically active form of allysine having L-configuration. | 3.62 | 2 | 0 | allysine; amino acid zwitterion; aminoadipate semialdehyde; non-proteinogenic L-alpha-amino acid | human metabolite; mouse metabolite; Saccharomyces cerevisiae metabolite |
| isobutyryl-1-carnitine O-isobutyryl-L-carnitine : An optically active form of O-isobutyrylcarnitine having (R)-configuration. | 2.44 | 2 | 0 | methyl-branched fatty acyl-L-carnitine; O-isobutyrylcarnitine; saturated fatty acyl-L-carnitine | human metabolite |
| 3-hydroxyglutaric acid 3-hydroxyglutaric acid : A 3 hydroxy carboxylic acid that is glutaric acid which is substituted by a hydroxy group at position 3. It is a diagnostic marker for glutaric aciduria type I. | 2.96 | 4 | 0 | 3-hydroxy carboxylic acid; alpha,omega-dicarboxylic acid | human blood serum metabolite; human urinary 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. | 7.41 | 1 | 0 | O-butanoylcarnitine; saturated fatty acyl-L-carnitine | metabolite |
| tretinoin Tretinoin: An important regulator of GENE EXPRESSION during growth and development, and in NEOPLASMS. Tretinoin, also known as retinoic acid and derived from maternal VITAMIN A, is essential for normal GROWTH; and EMBRYONIC DEVELOPMENT. An excess of tretinoin can be teratogenic. It is used in the treatment of PSORIASIS; ACNE VULGARIS; and several other SKIN DISEASES. It has also been approved for use in promyelocytic leukemia (LEUKEMIA, PROMYELOCYTIC, ACUTE).. retinoic acid : A retinoid consisting of 3,7-dimethylnona-2,4,6,8-tetraenoic acid substituted at position 9 by a 2,6,6-trimethylcyclohex-1-en-1-yl group (geometry of the four exocyclic double bonds is not specified).. all-trans-retinoic acid : A retinoic acid in which all four exocyclic double bonds have E- (trans-) geometry. | 2.17 | 1 | 0 | retinoic acid; vitamin A | anti-inflammatory agent; antineoplastic agent; antioxidant; AP-1 antagonist; human metabolite; keratolytic drug; retinoic acid receptor agonist; retinoid X receptor agonist; signalling molecule |
| alitretinoin Alitretinoin: A retinoid that is used for the treatment of chronic hand ECZEMA unresponsive to topical CORTICOSTEROIDS. It is also used to treat cutaneous lesions associated with AIDS-related KAPOSI SARCOMA. | 2.17 | 1 | 0 | retinoic acid | antineoplastic agent; keratolytic drug; metabolite; retinoid X receptor agonist |
| lysophosphatidylcholines lysophosphatidylcholine : An acylglycerophosphocholine resulting from partial hydrolysis of a phosphatidylcholine, which removes one of the fatty acyl groups. The structure is depicted in the image where R1 = acyl, R2 = H or where R1 = H, R2 = acyl. | 2.17 | 1 | 0 | 1-O-acyl-sn-glycero-3-phosphocholine | |
| 3-methylbutyrylcarnitine 3-methylbutyrylcarnitine: affects 2-oxo acid dehydrogenase activity in intact mitochondria of rat muscle; RN given refers to (R)-isomer. O-isovalerylcarnitine : A C5-acylcarnitine having isovaleryl as the acyl substituent. | 1.97 | 1 | 0 | C5-acylcarnitine | human metabolite |
| octanoylcarnitine octanoylcarnitine: RN given refers to cpd without isomeric designation. O-octanoyl-L-carnitine : The L-enantiomer of an O-octanoylcarnitine. | 2.05 | 1 | 0 | O-octanoylcarnitine; saturated fatty acyl-L-carnitine | human metabolite |
| palmitoylcarnitine Palmitoylcarnitine: A long-chain fatty acid ester of carnitine which facilitates the transfer of long-chain fatty acids from cytoplasm into mitochondria during the oxidation of fatty acids.. O-palmitoyl-L-carnitine : An O-acyl-L-carnitine in which the acyl group is specified as palmitoyl (hexadecanoyl). | 2.05 | 1 | 0 | O-palmitoylcarnitine; saturated fatty acyl-L-carnitine | EC 3.6.3.9 (Na(+)/K(+)-transporting ATPase) inhibitor; human metabolite; mouse metabolite |
| azaspiracid azaspiracid: a toxin from mussels produced in Ireland that caused multiple organ damage; structure in first source | 2.11 | 1 | 0 | ||
| transforming growth factor beta Transforming Growth Factor beta: A factor synthesized in a wide variety of tissues. It acts synergistically with TGF-alpha in inducing phenotypic transformation and can also act as a negative autocrine growth factor. TGF-beta has a potential role in embryonal development, cellular differentiation, hormone secretion, and immune function. TGF-beta is found mostly as homodimer forms of separate gene products TGF-beta1, TGF-beta2 or TGF-beta3. Heterodimers composed of TGF-beta1 and 2 (TGF-beta1.2) or of TGF-beta2 and 3 (TGF-beta2.3) have been isolated. The TGF-beta proteins are synthesized as precursor proteins. | 2.31 | 1 | 0 |
| Condition | Indicated | Relationship Strength | Studies | Trials |
|---|---|---|---|---|
| 2019 Novel Coronavirus Disease [description not available] | 0 | 2.31 | 1 | 0 |
| Amino Acid Metabolism Disorders, Inborn [description not available] | 0 | 5.1 | 16 | 0 |
| Brain Disorders [description not available] | 0 | 2.31 | 1 | 0 |
| Acquired Metabolic Diseases, Brain [description not available] | 0 | 4.74 | 10 | 0 |
| Dyskinesia Syndromes [description not available] | 0 | 2.31 | 1 | 0 |
| Decreased Muscle Tone [description not available] | 0 | 2.48 | 2 | 0 |
| Brain Diseases Pathologic conditions affecting the BRAIN, which is composed of the intracranial components of the CENTRAL NERVOUS SYSTEM. This includes (but is not limited to) the CEREBRAL CORTEX; intracranial white matter; BASAL GANGLIA; THALAMUS; HYPOTHALAMUS; BRAIN STEM; and CEREBELLUM. | 0 | 2.31 | 1 | 0 |
| Movement Disorders Syndromes which feature DYSKINESIAS as a cardinal manifestation of the disease process. Included in this category are degenerative, hereditary, post-infectious, medication-induced, post-inflammatory, and post-traumatic conditions. | 0 | 2.31 | 1 | 0 |
| Aura [description not available] | 0 | 3.17 | 1 | 0 |
| Diseases, Metabolic [description not available] | 0 | 3.43 | 2 | 0 |
| Brain Diseases, Metabolic, Familial [description not available] | 0 | 3.17 | 1 | 0 |
| Epilepsy A disorder characterized by recurrent episodes of paroxysmal brain dysfunction due to a sudden, disorderly, and excessive neuronal discharge. Epilepsy classification systems are generally based upon: (1) clinical features of the seizure episodes (e.g., motor seizure), (2) etiology (e.g., post-traumatic), (3) anatomic site of seizure origin (e.g., frontal lobe seizure), (4) tendency to spread to other structures in the brain, and (5) temporal patterns (e.g., nocturnal epilepsy). (From Adams et al., Principles of Neurology, 6th ed, p313) | 0 | 3.17 | 1 | 0 |
| Metabolic Diseases Generic term for diseases caused by an abnormal metabolic process. It can be congenital due to inherited enzyme abnormality (METABOLISM, INBORN ERRORS) or acquired due to disease of an endocrine organ or failure of a metabolically important organ such as the liver. (Stedman, 26th ed) | 0 | 3.43 | 2 | 0 |
| Innate Inflammatory Response [description not available] | 0 | 2.31 | 1 | 0 |
| Inflammation A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function. | 0 | 2.31 | 1 | 0 |
| Age-Related Osteoporosis [description not available] | 0 | 2.17 | 1 | 0 |
| Osteoporosis Reduction of bone mass without alteration in the composition of bone, leading to fractures. Primary osteoporosis can be of two major types: postmenopausal osteoporosis (OSTEOPOROSIS, POSTMENOPAUSAL) and age-related or senile osteoporosis. | 0 | 2.17 | 1 | 0 |
| Disease Models, Animal Naturally-occurring or experimentally-induced animal diseases with pathological processes analogous to human diseases. | 0 | 2.21 | 1 | 0 |
| Food Poisoning [description not available] | 0 | 2.11 | 1 | 0 |
| Kidney Failure A severe irreversible decline in the ability of kidneys to remove wastes, concentrate URINE, and maintain ELECTROLYTE BALANCE; BLOOD PRESSURE; and CALCIUM metabolism. | 0 | 2.05 | 1 | 0 |
| Renal Insufficiency Conditions in which the KIDNEYS perform below the normal level in the ability to remove wastes, concentrate URINE, and maintain ELECTROLYTE BALANCE; BLOOD PRESSURE; and CALCIUM metabolism. Renal insufficiency can be classified by the degree of kidney damage (as measured by the level of PROTEINURIA) and reduction in GLOMERULAR FILTRATION RATE. | 0 | 2.05 | 1 | 0 |
| Metabolic Acidosis [description not available] | 0 | 2.01 | 1 | 0 |
| Muscle Spasm [description not available] | 0 | 2.01 | 1 | 0 |
| Acidosis A pathologic condition of acid accumulation or depletion of base in the body. The two main types are RESPIRATORY ACIDOSIS and metabolic acidosis, due to metabolic acid build up. | 0 | 2.01 | 1 | 0 |
| Acute Disease Disease having a short and relatively severe course. | 0 | 2.01 | 1 | 0 |
| Atrophy Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes. | 0 | 2.01 | 1 | 0 |
| Spasm An involuntary contraction of a muscle or group of muscles. Spasms may involve SKELETAL MUSCLE or SMOOTH MUSCLE. | 0 | 2.01 | 1 | 0 |
| Sensitivity and Specificity Binary classification measures to assess test results. Sensitivity or recall rate is the proportion of true positives. Specificity is the probability of correctly determining the absence of a condition. (From Last, Dictionary of Epidemiology, 2d ed) | 0 | 2.02 | 1 | 0 |
| Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. | 0 | 1.97 | 1 | 0 |