Methylsuccinic acid is a normal metabolite found in human fluids and is an intermediate metabolite in the breakdown of fatty acids. Increased urinary levels of methylsuccinic acid (together with ethylmalonic acid) are the main biochemical measurable features in ethylmalonic encephalopathy.
References:
Urpi-Sarda M, Almanza-Aguilera E, Llorach R, Vázquez-Fresno R, Estruch R, Corella D, Sorli JV, Carmona F, Sanchez-Pla A, Salas-Salvadó J, Andres-Lacueva C. Non-targeted metabolomic biomarkers and metabotypes of type 2 diabetes: A cross-sectional study of PREDIMED trial participants. Diabetes Metab. 2019 Apr;45(2):167-174. doi: 10.1016/j.diabet.2018.02.006. Epub 2018 Feb 20. PMID: 29555466.
Tavasoli AR, Rostami P, Ashrafi MR, Karimzadeh P. Neurological and Vascular Manifestations of Ethylmalonic Encephalopathy. Iran J Child Neurol. 2017 Spring;11(2):57-60. PMID: 28698729; PMCID: PMC5493831.
Barth M, Ottolenghi C, Hubert L, Chrétien D, Serre V, Gobin S, Romano S, Vassault A, Sefiani A, Ricquier D, Boddaert N, Brivet M, de Keyzer Y, Munnich A, Duran M, Rabier D, Valayannopoulos V, de Lonlay P. Multiple sources of metabolic disturbance in ETHE1-related ethylmalonic encephalopathy. J Inherit Metab Dis. 2010 Dec;33 Suppl 3:S443-53. doi: 10.1007/s10545-010-9227-y. Epub 2010 Oct 27. PMID: 20978941.
Grosso S, Mostardini R, Farnetani MA, Molinelli M, Berardi R, Dionisi-Vici C, Rizzo C, Morgese G, Balestri P. Ethylmalonic encephalopathy: further clinical and neuroradiological characterization. J Neurol. 2002 Oct;249(10):1446-50. doi: 10.1007/s00415-002-0880-4. PMID: 12382164.
Gallant NM, Leydiker K, Tang H, Feuchtbaum L, Lorey F, Puckett R, Deignan JL, Neidich J, Dorrani N, Chang E, Barshop BA, Cederbaum SD, Abdenur JE, Wang RY. Biochemical, molecular, and clinical characteristics of children with short chain acyl-CoA dehydrogenase deficiency detected by newborn screening in California. Mol Genet Metab. 2012 May;106(1):55-61. doi: 10.1016/j.ymgme.2012.02.007. Epub 2012 Feb 9. PMID: 22424739.
Tein I, Elpeleg O, Ben-Zeev B, Korman SH, Lossos A, Lev D, Lerman-Sagie T, Leshinsky-Silver E, Vockley J, Berry GT, Lamhonwah AM, Matern D, Roe CR, Gregersen N. Short-chain acyl-CoA dehydrogenase gene mutation (c.319C>T) presents with clinical heterogeneity and is candidate founder mutation in individuals of Ashkenazi Jewish origin. Mol Genet Metab. 2008 Feb;93(2):179-89. doi: 10.1016/j.ymgme.2007.09.021. Epub 2007 Dec 3. PMID: 18054510.
Bhala A, Willi SM, Rinaldo P, Bennett MJ, Schmidt-Sommerfeld E, Hale DE. Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency. J Pediatr. 1995 Jun;126(6):910-5. doi: 10.1016/s0022-3476(95)70207-5. PMID: 7776094.
Goodman SI, McCabe ER, Fennessey PV, Mace JW. Multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type II) with transient hypersarcosinemia and sarcosinuria; possible inherited deficiency of an electron transfer flavoprotein. Pediatr Res. 1980 Jan;14(1):12-7. doi: 10.1203/00006450-198001000-00004. PMID: 7360517.
Tojo M, Gunji T, Yamaguchi S, Shimizu N, Koga Y, Nonaka I. [A case of riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type II)]. No To Hattatsu. 2000 Mar;32(2):163-8. Japanese. PMID: 10723193.
Gregersen N, Wintzensen H, Christensen SK, Christensen MF, Brandt NJ, Rasmussen K. C6-C10-dicarboxylic aciduria: investigations of a patient with riboflavin responsive multiple acyl-CoA dehydrogenation defects. Pediatr Res. 1982 Oct;16(10):861-8. doi: 10.1203/00006450-198210000-00012. PMID: 7145508.
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Loots DT. Abnormal tricarboxylic acid cycle metabolites in isovaleric acidaemia. J Inherit Metab Dis. 2009 Jun;32(3):403-11. doi: 10.1007/s10545-009-1071-6. Epub 2009 Apr 5. PMID: 19343532.
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Increased levels of methylsuccinate may occur when there are nutritional enzyme inhibitions of the breakdown pathways, inherited low-activity enzymes are present, or if there are high levels of precursors (ethylmalonate).
Methylsuccinate is normally taken up by pancreatic beta-cells where it stimulates insulin release; high levels may indicate a problem with the pancreas or with cellular uptake mechanisms.
Obesity may increase methylsuccinate levels. If liver beta-oxidation of fatty acids is impaired methylsuccinate can increase. Mobilization of stored body fat during fasting or calorie restriction may also increase ethylmalonate and methylsuccinate levels. High- fat or -protein diets, or the use of branch-chain amino acid supplements, may also increase methylmalonate levels.
→ Consider supporting liver beta-oxidation with vitamins B2, B3, iron (if deficient), L-carnitine, sulforaphane and resveratrol. Low activity enzyme variants may benefit from a lower protein or lower fat diet. If using a high-protein diet or branch-chain amino acids consider vitamins B1, B2, B3, B6, and lipoic acid to support their breakdown.
→ Type II diabetes or metabolic syndrome can increase suberate, adipate, alpha-hydroxybutyrate, lactate, and pyruvate.
→ Phthalate exposures can inhibit liver beta-oxidation and increase levels of adipate, suberate, ethylmalonate, and methylsuccinate.
Increased urinary levels of methylsuccinic acid (together with ethylmalonic acid) are the main biochemical measurable features in ethylmalonic encephalopathy, a rare metabolic disorder with an autosomal recessive mode of inheritance that is clinically characterized by neuromotor delay, hyperlactic acidemia, recurrent petechiae, orthostatic acrocyanosis, and chronic diarrhea. The underlying biochemical defect involves isoleucine catabolism.
Other inborn errors:
Moreover, methylsuccinic acid is found to be associated with isovaleric acidemia, and medium-chain acyl-CoA dehydrogenase deficiency, which are also inborn errors of metabolism.
High Methylsuccinic levels may also be due to:
- carnitine deficiency,
- fasting,
- increased intake of the medium-chain triglycerides found in coconut oil, MCT oil, and some infant formulas.
Regardless of cause, supplementation with L-carnitine or acetyl-L-carnitine (500-1000 mg per day) may be beneficial.
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Low levels of methylsuccinate can occur if there are low levels of precursors (ethylmalonate), if there are nutritional enzyme inhibitions, or if a low-activity enzyme variant is inherited.
Methylsuccinate is taken up by pancreatic beta-cells to stimulate the release of insulin. Methylsuccinate uptake by the pancreas and other cells may protect against mitochondrial damage from biguanide medications (metformin, etc.) often given to diabetic patients.
Methylsuccinate, like ethylmalonate, is primarily derived from branch-chain fatty acids. Low levels of branch-chain fatty acids (dairy, beef) may result in lower methylsuccinate levels.
→ Consider supporting methylsuccinate synthesis with adenosyl-cobalamin (B12), biotin, magnesium, and L-carnitine. Increasing healthy fats or proteins in the diet may also increase methylmalonate levels. If B-12 is deficient, methylmalonate and ethylmalonate may be high.
→ Methylmalonate is derived primarily from the amino acid valine. If alpha- ketoisovalerate is high, there may be a downstream block that prevents the formation of methylmalonate, and levels may be lower than expected even if there is a vitamin B-12 deficiency present.
→ Succinate cannot be methylated if there are enzyme inhibitions that prevent the synthesis of methyl groups. Problems on the methylation pathway may increase levels of ethylmalonate, methylmalonate, 5-hydroxyindoleacetate and decrease levels of homovanillate and vanilmandelate.
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Optimal Result:
0.13 - 2.14 mmol/mol.