Gene Association: MMAB
UniProt Search:
MMAB (PROTEIN_CODING)
Function Description: metabolism of cobalamin associated B
found 24 associated metabolites with current gene based on the text mining result from the pubmed database.
Methylmalonyl-CoA
Methylmalonyl-CoA is an intermediate in the metabolism of Propanoate. It is a substrate for Malonyl-CoA decarboxylase (mitochondrial), Methylmalonyl-CoA mutase (mitochondrial) and Methylmalonyl-CoA epimerase (mitochondrial). [HMDB] Methylmalonyl-CoA is an intermediate in the metabolism of Propanoate. It is a substrate for Malonyl-CoA decarboxylase (mitochondrial), Methylmalonyl-CoA mutase (mitochondrial) and Methylmalonyl-CoA epimerase (mitochondrial).
Methylmalonic acid
Methylmalonic acid is a malonic acid derivative, which is a vital intermediate in the metabolism of fat and protein. In particular, the coenzyme A-linked form of methylmalonic acid, methylmalonyl-CoA, is converted into succinyl-CoA by methylmalonyl-CoA mutase in a reaction that requires vitamin B12 as a cofactor. In this way, methylmalonic acid enters the Krebs cycle and is thus part of one of the anaplerotic reactions. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This inborn error of metabolism is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA. Methylmalonic acid is also found to be associated with other inborn errors of metabolism, including cobalamin deficiency, cobalamin malabsorption, malonyl-CoA decarboxylase deficiency, and transcobalamin II deficiency. When present in sufficiently high levels, methylmalonic acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of methylmalonic acid are associated with at least 5 inborn errors of metabolism, including Malonyl CoA decarboxylase deficiency, Malonic Aciduria, Methylmalonate Semialdehyde Dehydrogenase Deficiency, Methylmalonic Aciduria and Methylmalonic Aciduria Due to Cobalamin-Related Disorders. Methylmalonic acid is an organic acid and abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart abnormalities, kidney abnormalities, liver damage, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures. 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. [HMDB] KEIO_ID M014 Methylmalonic acid (Methylmalonate) is an indicator of Vitamin B-12 deficiency in cancer. Methylmalonic acid (Methylmalonate) is an indicator of Vitamin B-12 deficiency in cancer.
Propionylcarnitine
D018373 - Peripheral Nervous System Agents > D018689 - Sensory System Agents An O-acylcarnitine compound having propanoyl as the acyl substituent. D002491 - Central Nervous System Agents > D000700 - Analgesics D020011 - Protective Agents > D002316 - Cardiotonic Agents D000893 - Anti-Inflammatory Agents D002317 - Cardiovascular Agents D018501 - Antirheumatic Agents
Cobamamide
A member of the class of cobalamins that is vitamin B12 in which the cyano group is replaced by a 5-deoxyadenos-5-yl moiety. It is one of the two metabolically active form of vitamin B12. B - Blood and blood forming organs > B03 - Antianemic preparations > B03B - Vitamin b12 and folic acid > B03BA - Vitamin b12 (cyanocobalamin and analogues) Adenosylcobalamin (Coenzyme B12;Cobamamide;AdoCbl) is an active form of Vitamin B12 which is a cofactor for methylmalonyl CoA mutase[1] Adenosylcobalamin (Coenzyme B12;Cobamamide;AdoCbl) is an active form of Vitamin B12 which is a cofactor for methylmalonyl CoA mutase[1]
Succinyl-CoA
Succinyl-CoA is an important intermediate in the citric acid cycle, where it is synthesized from α-Ketoglutarate by α-ketoglutarate dehydrogenase (EC 1.2.4.2) through decarboxylation, and is converted into succinate through the hydrolytic release of coenzyme A by succinyl-CoA synthetase (EC 6.2.1.5). Succinyl-CoA may be an end product of peroxisomal beta-oxidation of dicarboxylic fatty acids; the identification of an apparently specific succinyl-CoA thioesterase (ACOT4, EC 3.1.2.3, hydrolyzes succinyl-CoA) in peroxisomes strongly suggests that succinyl-CoA is formed in peroxisomes. Acyl-CoA thioesterases (ACOTs) are a family of enzymes that catalyze the hydrolysis of the CoA esters of various lipids to the free acids and coenzyme A, thereby regulating levels of these compounds. (PMID: 16141203) [HMDB]. Succinyl-CoA is found in many foods, some of which are fruits, sea-buckthornberry, pomegranate, and sweet orange. Succinyl-CoA is an important intermediate in the citric acid cycle, where it is synthesized from α-Ketoglutarate by α-ketoglutarate dehydrogenase (EC 1.2.4.2) through decarboxylation, and is converted into succinate through the hydrolytic release of coenzyme A by succinyl-CoA synthetase (EC 6.2.1.5). Succinyl-CoA may be an end product of peroxisomal beta-oxidation of dicarboxylic fatty acids; the identification of an apparently specific succinyl-CoA thioesterase (ACOT4, EC 3.1.2.3, hydrolyzes succinyl-CoA) in peroxisomes strongly suggests that succinyl-CoA is formed in peroxisomes. Acyl-CoA thioesterases (ACOTs) are a family of enzymes that catalyze the hydrolysis of the CoA esters of various lipids to the free acids and coenzyme A, thereby regulating levels of these compounds. (PMID: 16141203).
Mevalonic acid-5P
Mevalonic acid-5p, also known as (R)-5-phosphomevalonate or mevalonate-5p, is a member of the class of compounds known as monoalkyl phosphates. Monoalkyl phosphates are organic compounds containing a phosphate group that is linked to exactly one alkyl chain. Thus, mevalonic acid-5p is considered to be a fatty acid lipid molecule. Mevalonic acid-5p is soluble (in water) and a moderately acidic compound (based on its pKa). Mevalonic acid-5p can be found in a number of food items such as rowanberry, common oregano, caraway, and cherry tomato, which makes mevalonic acid-5p a potential biomarker for the consumption of these food products. Mevalonic acid-5p can be found primarily throughout most human tissues. Mevalonic acid-5p exists in all eukaryotes, ranging from yeast to humans. In humans, mevalonic acid-5p is involved in several metabolic pathways, some of which include pamidronate action pathway, rosuvastatin action pathway, pravastatin action pathway, and lovastatin action pathway. Mevalonic acid-5p is also involved in several metabolic disorders, some of which include hypercholesterolemia, lysosomal acid lipase deficiency (wolman disease), hyper-igd syndrome, and mevalonic aciduria. Mevalonic acid-5P (CAS: 1189-94-2), also known as 5-phosphomevalonic acid, belongs to the class of organic compounds known as monoalkyl phosphates. These are organic compounds containing a phosphate group that is linked to exactly one alkyl chain. Within humans, mevalonic acid-5P participates in many enzymatic reactions. In particular, mevalonic acid-5P can be biosynthesized from mevalonate; which is mediated by the enzyme mevalonate kinase. In addition, mevalonic acid-5P can be converted into mevalonic acid-5-pyrophosphate through its interaction with the enzyme phosphomevalonate kinase. In humans, mevalonic acid-5P is involved in the mevalonate pathway. Outside of the human body, mevalonic acid-5P has been detected, but not quantified in, several different foods, such as oriental wheat, devilfish, pepper (spice), redcurrants, and star fruits. This could make mevalonic acid-5P a potential biomarker for the consumption of these foods.
FMNH2
FMNH2 is the reduced form of flavin mononucleotide. It is a substrate of the enzyme FMN reductase (EC 1.5.1.29), an enzyme that catalyzes the chemical reaction FMNH2 + NAD(P)+ <=> FMN + NAD(P)H + H+. Flavin mononucleotide (FMN), or riboflavin-5′-phosphate, is a biomolecule produced from riboflavin (vitamin B2) by the enzyme riboflavin kinase and functions as prosthetic group of various oxidoreductases including NADH dehydrogenase. During a catalytic cycle, the reversible interconversion of oxidized (FMN), semiquinone (FMNH•) and reduced (FMNH2) forms occurs in the various oxidoreductases. FMN is a stronger oxidizing agent than NAD and is particularly useful because it can take part in both one- and two-electron transfers. FMNH is a substrate for 2,4-dienoyl-CoA reductase (mitochondrial). [HMDB]
Propylene glycol
Propylene glycol (CAS: 57-55-6), also known as 1,2-propanediol, is an organic compound (a diol alcohol), usually a tasteless, odourless, and colourless clear oily liquid that is hygroscopic and miscible with water, acetone, and chloroform. It is manufactured by the hydration of propylene oxide. Propylene glycol is used as a solvent for intravenous, oral, and topical pharmaceutical preparations It is generally considered safe. However, in large doses, it can be toxic, especially if given over a short period of time. Intravenous lorazepam contains the largest amount of propylene glycol of commonly used drugs. In adults with normal liver and kidney function, the terminal half-life of propylene glycol ranges from 1.4 to 3.3 hours. Propylene glycol is metabolized by the liver to form lactate, acetate, and pyruvate. The nonmetabolized drug is excreted in the urine mainly as the glucuronide conjugate, approximately 12 to 45 percent is excreted unchanged in urine. Renal clearance decreases as the dose administered increases (390 ml/minute/173 m2 at a dose of 5 g/day but only 144 ml/minute/173 m2 at a dose of 21 g/day). These data suggest that renal clearance declines at higher propylene glycol doses because of the saturation of proximal tubular secretion of the drug. As an acceptable level of propylene glycol has not been defined, the clinical implication of a propylene glycol level is unclear. The World Health Organization (WHO) recommends a maximum consumption of 25 mg/kg/day (1.8 g/day for a 75 kg male) of propylene glycol when used as a food additive, but this limit does not address its use as a drug solvent. No maximum dose is recommended in the literature for intravenous therapy with propylene glycol. Intoxication occurs at much higher doses than the WHO dose limit and is exclusive to pharmacologic exposure. Propylene glycol toxicity includes the development of serum hyperosmolality, lactic acidosis, and kidney failure. It has been suggested that proximal tubular necrosis is the cause of acute kidney injury from propylene glycol. Along these lines, proximal tubular cell injury occurs in cultured human cells exposed to propylene glycol. Acute tubular necrosis was described with propylene glycol toxicity in a case of concomitant administration of intravenous lorazepam and trimethoprim sulfamethoxazole. Propylene glycol induced intoxication can also mimic sepsis or systemic inflammatory response syndrome (SIRS). Patients suspected of having sepsis with negative cultures should be evaluated for propylene glycol toxicity if they have been exposed to high dose lorazepam or other medications containing this solvent (PMID:17555487). Propylene glycol is an anticaking agent, antioxidant, dough strengthener, emulsifier, flavouring agent, formulation aid, humectant, solvent, preservative, stabiliser, hog/poultry scald agent, and surface active agent. It is found in foods such as roasted sesame seeds, oats, truffle and other mushrooms. (R)-(-)-1,2-Propanediol is a (R)-enantiomer of 1,2-Propanediol that produced from glucose in Escherichia coli expressing NADH-linked glycerol dehydrogenase genes[1]. (R)-(-)-1,2-Propanediol is a (R)-enantiomer of 1,2-Propanediol that produced from glucose in Escherichia coli expressing NADH-linked glycerol dehydrogenase genes[1].
4-O-(Indole-3-acetyl)-D-glucopyranose
4-o-(indole-3-acetyl)-d-glucopyranose, also known as indole-3-acetyl-beta-1-D-glucose or B-D-glucopyranose, 1-(1h-indole-3-acetic acid), belongs to indole-3-acetic acid derivatives class of compounds. Those are compounds containing an acetic acid (or a derivative) linked to the C3 carbon atom of an indole. 4-o-(indole-3-acetyl)-d-glucopyranose is slightly soluble (in water) and a very weakly acidic compound (based on its pKa). 4-o-(indole-3-acetyl)-d-glucopyranose can be found in corn, which makes 4-o-(indole-3-acetyl)-d-glucopyranose a potential biomarker for the consumption of this food product. D006133 - Growth Substances > D010937 - Plant Growth Regulators > D007210 - Indoleacetic Acids
Tenofovir disoproxil
J - Antiinfectives for systemic use > J05 - Antivirals for systemic use > J05A - Direct acting antivirals > J05AF - Nucleoside and nucleotide reverse transcriptase inhibitors D000890 - Anti-Infective Agents > D000998 - Antiviral Agents > D018894 - Reverse Transcriptase Inhibitors D000890 - Anti-Infective Agents > D000998 - Antiviral Agents > D044966 - Anti-Retroviral Agents COVID info from clinicaltrial, clinicaltrials, clinical trial, clinical trials D004791 - Enzyme Inhibitors > D019384 - Nucleic Acid Synthesis Inhibitors Corona-virus Coronavirus SARS-CoV-2 COVID-19 SARS-CoV COVID19 SARS2 SARS
propylene glycol
D010592 - Pharmaceutic Aids > D014677 - Pharmaceutical Vehicles D012997 - Solvents (R)-(-)-1,2-Propanediol is a (R)-enantiomer of 1,2-Propanediol that produced from glucose in Escherichia coli expressing NADH-linked glycerol dehydrogenase genes[1]. (R)-(-)-1,2-Propanediol is a (R)-enantiomer of 1,2-Propanediol that produced from glucose in Escherichia coli expressing NADH-linked glycerol dehydrogenase genes[1].
CoA 4:1;O2
The (R)-enantiomer of methylmalonyl-CoA.
S-Methylmalonyl-CoA
Methylmalonyl-CoA is an intermediate in the metabolism of Propanoate. It is a substrate for Malonyl-CoA decarboxylase (mitochondrial), Methylmalonyl-CoA mutase (mitochondrial) and Methylmalonyl-CoA epimerase (mitochondrial). [HMDB] Methylmalonyl-CoA is an intermediate in the metabolism of Propanoate. It is a substrate for Malonyl-CoA decarboxylase (mitochondrial), Methylmalonyl-CoA mutase (mitochondrial) and Methylmalonyl-CoA epimerase (mitochondrial).
Methylmalonic acid
A dicarboxylic acid that is malonic acid in which one of the methylene hydrogens is substituted by a methyl group. Methylmalonic acid (Methylmalonate) is an indicator of Vitamin B-12 deficiency in cancer. Methylmalonic acid (Methylmalonate) is an indicator of Vitamin B-12 deficiency in cancer.
CAR 3:0
D018373 - Peripheral Nervous System Agents > D018689 - Sensory System Agents D002491 - Central Nervous System Agents > D000700 - Analgesics D020011 - Protective Agents > D002316 - Cardiotonic Agents D000893 - Anti-Inflammatory Agents D002317 - Cardiovascular Agents D018501 - Antirheumatic Agents
(3R)-3-Hydroxy-3-methyl-5-(phosphonooxy)pentanoic acid
1-Deoxy-1-(7,8-dimethyl-2,4-dioxo-3,4-dihydro-2H-benzo[G]pteridin-1-ID-10(5H)-YL)-5-O-phosphonato-D-ribitol
1-O-indol-3-Ylacetyl-beta-D-glucose
D006133 - Growth Substances > D010937 - Plant Growth Regulators > D007210 - Indoleacetic Acids
