Target intelligence / Profile preview

Electron transfer flavoprotein beta subunit lysine methyltransferase (ETFBKMT)

Target
ETFBKMT
Molecular classification
Enzyme, Protein-lysine methyltransferase, Mitochondrial protein
01

Overview

Electron transfer flavoprotein beta subunit lysine methyltransferase (ETFBKMT, also known as METTL20) is a mitochondrial enzyme that specifically trimethylates lysine residues on the beta subunit of electron transfer flavoprotein (ETFB). This posttranslational modification can negatively regulate ETFB’s function in electron transfer from acyl-CoA dehydrogenases to the respiratory chain, thus modulating mitochondrial fatty acid oxidation and energy metabolism. Genetic variants in ETFBKMT are associated with metabolic disorders such as multiple acyl-CoA dehydrogenase deficiency, which impacts mitochondrial fatty acid and amino acid catabolism. The protein is also involved in pathways related to protein methylation and has a paralog, HEMK1. Currently, no direct drugs are approved or known to target ETFBKMT, and there are no established biomarkers or safety issues specifically associated with this enzyme in clinical contexts.

Other names
METTL20C12orf72ETKMTProtein N-lysine methyltransferase METTL20Methyltransferase-like protein 20ETFB lysine methyltransferaseDKFZp451L235MGC50559
02

Mechanism of action

Catalytic methylation of lysine residues on ETFB, regulating its function in the mitochondrial electron transfer chain

03

Biological functions

Trimethylation of electron transfer flavoprotein beta subunit (ETFB)Regulation of mitochondrial electron transferNegative regulation of fatty acid beta-oxidationParticipation in protein methylation pathways
04

Disease associations

Multiple acyl-CoA dehydrogenase deficiencyKallmann syndrome

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