16284-60-9

Usage

Uses

Used in Diagnostic Applications:
N-Isovalerylglycine is used as a diagnostic tool for identifying and monitoring various organic acidemias and mitochondrial fatty acid β-oxidation defects. It is particularly useful in the detection of isovaleric acidemia, an inborn error of leucine metabolism.
Used in Medical Industry:
N-Isovalerylglycine is used as a diagnostic marker in the medical industry for the early detection and diagnosis of isovaleric acidemia and other related metabolic disorders. Its presence in the urine of affected patients aids in the identification of these conditions, enabling timely intervention and treatment.
Used in Research and Development:
N-Isovalerylglycine is also utilized in research and development for the study of metabolic pathways, enzyme deficiencies, and the development of novel therapeutic strategies for the treatment of isovaleric acidemia and other related metabolic disorders.

InChI:InChI=1/C7H13NO3/c1-5(2)3-6(9)8-4-7(10)11/h5H,3-4H2,1-2H3,(H,8,9)(H,10,11)

Upstream product

• 56-40-6 glycine 
• 108-12-3 isopentanoyl chloride 
• 503-74-2 3-methylbutyric acid 
• 6244-91-3 isovaleryl-CoA 

Relevant academic research and scientific papers

Repurposing the 3-Isocyanobutanoic Acid Adenylation Enzyme SfaB for Versatile Amidation and Thioesterification

Genome mining of microbial natural products enables chemists not only to discover the bioactive molecules with novel skeletons, but also to identify the enzymes that catalyze diverse chemical reactions. Exploring the substrate promiscuity and catalytic mechanism of those biosynthetic enzymes facilitates the development of potential biocatalysts. SfaB is an acyl adenylate-forming enzyme that adenylates a unique building block, 3-isocyanobutanoic acid, in the biosynthetic pathway of the diisonitrile natural product SF2768 produced by Streptomyces thioluteus, and this AMP-ligase was demonstrated to accept a broad range of short-chain fatty acids (SCFAs). Herein, we repurpose SfaB to catalyze amidation or thioesterification between those SCFAs and various amine or thiol nucleophiles, thereby providing an alternative enzymatic approach to prepare the corresponding amides and thioesters in vitro.

Enzymatic characterization and elucidation of the catalytic mechanism of a recombinant bovine glycine N-acyltransferase

Glycine conjugation, a phase II detoxification process, is catalyzed by glycine N-acyltransferase (GLYAT; E.C. 2.3.1.13). GLYAT detoxifies various xenobiotics, such as benzoic acid, and endogenous organic acids, such as isovaleric acid, which makes GLYAT important in the management of organic acidemias in humans. We cloned the open reading frame encoding the bovine ortholog of GLYAT from bovine liver mRNA into the bacterial expression vector pColdIII. The recombinant enzyme was expressed, partially purified, and enzymatically characterized. Protein modeling was used to predict Glu 226 of bovine GLYAT to be catalytically important. This was assessed by constructing an E226Q mutant and comparing its enzyme kinetics to that of the wild-type recombinant bovine GLYAT. The Michaelis constants for benzoyl-CoA and glycine were determined and were similar for wild-type recombinant GLYAT, E226Q recombinant GLYAT, and GLYAT present in bovine liver. At pH 8.0, the E226Q mutant GLYAT had decreased activity, which could be compensated for by increasing the reaction pH. This suggested a catalytic mechanism in which Glu226 functions to deprotonate glycine, facilitating nucleophilic attack on the acyl- CoA. The recombinant bovine GLYAT enzyme, combined with this new understanding of its active site and reaction mechanism, could be a powerful tool to investigate the functional significance of GLYAT sequence variations. Eventually, this should facilitate investigations into the impact of known and novel sequence variations in the human GLYAT gene. Copyright

Composition for enhancing lipid production, barrier function, hydrogen peroxide neutralization, and moisturization of the skin

Increased production of skin lipids, increased barrier function, hydrogen peroxide neutralization, prevention of loss of the natural moisturizing factor from the stratum corneum and moisturization of the skin is provided by a topically applicable composition which includes one or more components selected from the group consisting of branched chain amino acids, derivatives of branched chain amino acids and mixtures thereof, which one or more components are capable of being catabolized in epidermal cells to form lipid precursors for epidermal lipid synthesis. The composition can also include one or more enzyme activators which increase the rate of catabolism of the one or more components.

N-Acylglycines: Gas chromatographic mass spectrometric identification and determination in urine by selected ion monitoring

Eleven biologically interesting N-acylglycines have been synthesized and the gas chromatographic and mass spectrometric properties of their trimethylsilyl derivatives studied. A sharp and reproducible gas chromatographic peak could be obtained for each n-acylglycine as the N,O-bis(trimethylsilyl)-N-acylglycine. By the use of these derivatives a sensitive and specific selected ion monitoring method for the determination of N-acylglycines in human urine has been developed.