Details of Metabolite

General Information of MET (ID: META00451)
Name Methylsuccinic acid
Synonyms   Click to Show/Hide Synonyms of This Metabolite
(-)-(2S)-Methylbutanedioic acid; (-)-2-Methylsuccinic acid; (-)-Methylsuccinic acid; (2S)-2-Methylbutanedioic acid; (2S)-Methylbutanedioic acid; (S)-(-)-2-Methylsuccinic acid; (S)-(-)-Methylsuccinic acid; (S)-2-Methylbutanedioate; (S)-2-Methylbutanedioic acid; (S)-2-Methylsuccinic acid; (S)-Methylsuccinate; (S)-Methylsuccinic acid; 1,2-Propanedicarboxylic acid; 2-Methylbutane-1,4-dioic acid; 2-Methylbutanedioic acid; 2-Methylsuccinic acid; Methylbutanedioic acid; Methylsuccinic acid; Pyrotartaric acid
Source Endogenous;Fatty acyls;Food
Structure Type   Fatty acids and conjugates  (Click to Show/Hide the Complete Structure Type Hierarchy)
Lipids and lipid-like molecules
Fatty Acyls
Fatty acids and conjugates
PubChem CID
6950476
HMDB ID
HMDB0001844
Formula
C5H8O4
Structure
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3D MOL 2D MOL
  Click to Show/Hide the Molecular/Functional Data (External Links/Property/Function) of This Metabolite
ChEBI ID
91316
FooDB ID
FDB022704
ChemSpider ID
5323413
Physicochemical Properties Molecular Weight 132.11 Topological Polar Surface Area 74.6
XlogP -0.2 Complexity 129
Heavy Atom Count 9 Rotatable Bond Count 3
Hydrogen Bond Donor Count 2 Hydrogen Bond Acceptor Count 4
Function
Methylsuccinic acid (CAS: 498-21-5) is a normal metabolite found in human fluids. Increased urinary levels of methylsuccinic acid (together with ethylmalonic acid) are the main biochemical measurable features in ethylmalonic encephalopathy (OMIM: 602473), 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. Methylsuccinic acid levels were found to have decreased in the urine of animals under D-serine-induced nephrotoxicity (D-serine causes selective necrosis of the proximal straight tubules in the rat kidney). Moreover, methylsuccinic acid is found to be associated with ethylmalonic encephalopathy, isovaleric acidemia, and medium-chain acyl-CoA dehydrogenase deficiency, which are also inborn errors of metabolism.
Regulatory Network
Full List of Protein(s) Regulating This Metabolite
      Transcription factor (TF)
            R2R3-MYB (AN2) Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Protein   Info click to show the details of this protein
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Overexpression of AN2
                      Induced Change Methylsuccinic acid concentration: decrease (FC = 0.30)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Healthy individual
                      Details It is reported that overexpression of AN2 leads to the decrease of methylsuccinic acid levels compared with control group.
      Transferases (EC 2)
            Squalene synthase (FDFT1) Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Protein   Info click to show the details of this protein
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [2]
                      Introduced Variation Mutation of FDFT1
                      Induced Change Methylsuccinic acid concentration: increase
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lipid metabolism disorders [ICD-11: 5C52]
                      Details It is reported that mutation (patients with variants in FDFT1) of FDFT1 leads to the increase of methylsuccinic acid levels compared with control group.
References
1 Comprehensive Influences of Overexpression of a MYB Transcriptor Regulating Anthocyanin Biosynthesis on Transcriptome and Metabolome of Tobacco Leaves. Int J Mol Sci. 2019 Oct 16;20(20):5123.
2 Squalene Synthase Deficiency.

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