Details of Metabolite

General Information of MET (ID: META00528)
Name 3-Aminoisobutanoic acid
Synonyms   Click to Show/Hide Synonyms of This Metabolite
(+/-)-3-amino-2-methylpropanoate; (+/-)-3-amino-2-methylpropanoic acid; (+/-)-3-aminoisobutyric acid; (+/-)-b-aminoisobutyric acid; (+/-)-beta-aminoisobutyric acid; 2-(Aminomethyl)propionate; 2-(Aminomethyl)propionic acid; 2-Methyl-beta-alanine; 3-Amino-2-methylpropanoate; 3-Amino-2-methylpropanoic acid; 3-Amino-isobutanoate; 3-Amino-isobutanoic acid; 3-Aminoisobutanoate; 3-Aminoisobutanoic acid; 3-Aminoisobutyrate; 3-Aminoisobutyric acid; 3-Aminoisobutyric acid, (+-)-isomer; 3-Aminoisobutyric acid, (R)-isomer; 3-Aminoisobutyric acid, (S)-isomer; 3-Aminoisobutyric acid, tritium-labeled; BAIB; DL-2-Methyl-b-alanine; DL-2-Methyl-beta-alanine; DL-3-Amino-2-methylpropionic acid; DL-3-Aminoisobutyric acid; DL-3-Aminoisobutyric acid monohydrate; DL-b-Aminoisobutyrate; DL-b-Aminoisobutyric acid; DL-beta-Aminoisobutyric acid; alpha-Methyl-beta-alanine; b-Aminoisobutyrate; b-Aminoisobutyric acid; beta-Aminoisobutyric acid
Source Endogenous;Yeast Metabolite;Fatty acyls;Food;Microbial
Structure Type   Amino acids, peptides, and analogues  (Click to Show/Hide the Complete Structure Type Hierarchy)
Organic acids and derivatives
Carboxylic acids and derivatives
Amino acids, peptides, and analogues
PubChem CID
64956
HMDB ID
HMDB0003911
Formula
C4H9NO2
Structure
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3D MOL 2D MOL
  Click to Show/Hide the Molecular/Functional Data (External Links/Property/Function) of This Metabolite
KEGG ID
C05145
ChEBI ID
27389
FooDB ID
FDB023248
ChemSpider ID
58481
METLIN ID
5472
Physicochemical Properties Molecular Weight 103.12 Topological Polar Surface Area 63.3
XlogP -2.9 Complexity 72.1
Heavy Atom Count 7 Rotatable Bond Count 2
Hydrogen Bond Donor Count 2 Hydrogen Bond Acceptor Count 3
Function
beta-aminoisobutyric acid is the product from the conversion of N-carbamyl-beta-aminoisobutyric acid by the enzyme Beta-ureidopropionase (EC 3.5.1.6), the last step in pyrimidine degradation. Beta-ureidopropionase deficiency is an inborn error of pyrimidine degradation associated with neurological abnormalities (OMIM 606673).
Regulatory Network
Full List of Protein(s) Regulating This Metabolite
      Oxidoreductases (EC 1)
            Glyoxylate reductase/hydroxypyruvate reductase (GRHPR) 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 Mutation (patients: c.454dup (p.Thr152Asnfs*39)) of GRHPR
                      Induced Change 3-Aminoisobutanoic acid concentration: increase
                      Summary Introduced Variation         Induced Change 
                      Disease Status Carbohydrate metabolism disorders [ICD-11: 5C51]
                      Details It is reported that mutation (patients with c.454dup (p.Thr152Asnfs*39)) of GRHPR leads to the increase of 3-aminoisobutanoic acid levels compared with control group.
      Transferases (EC 2)
            Arylamine N-acetyltransferase 1 (NAT1) 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 Knockout of NAT1
                      Induced Change 3-Aminoisobutanoic acid concentration: decrease (FC = 0.3)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Breast cancer [ICD-11: 2C60]
                      Details It is reported that knockout of NAT1 leads to the decrease of 3-aminoisobutanoic acid levels compared with control group.
References
1 Severe child form of primary hyperoxaluria type 2 - a case report revealing consequence of GRHPR deficiency on metabolism. BMC Med Genet. 2017 May 31;18(1):59.
2 CRISPR/Cas9 knockout of human arylamine N-acetyltransferase 1 in MDA-MB-231 breast cancer cells suggests a role in cellular metabolism. Sci Rep. 2020 Jun 17;10(1):9804.

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