IDH3B: A Promising Drug Target for the Treatment of Mitochondrial Disorders

Target Name: IDH3B

NCBI ID: G3420

IDH3B

IDH3B: A Promising Drug Target for the Treatment of Mitochondrial Disorders

Mitochondrial disorders are a group of genetic disorders that affect the powerhouses of cells, leading to a range of progressive muscle weakness, liver disease, and other serious health problems. The most common form of mitochondrial disorders is called MELAS (Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like episodes), which affects approximately 10,000 people worldwide. Unfortunately, current treatment options for MELAS are limited, and there is no cure. IDH3B, a protein encoded by the nuclear gene IDH3 (Isoleucine-Dipeptidase [NAD] subunit beta), is a promising drug target for the treatment of mitochondrial disorders. In this article, we will discuss the current research on IDH3B, its potential as a drug target, and its potential as a biomarker for the diagnosis and prognosis of mitochondrial disorders.

The Importance of IDH3B

IDH3B is a key enzyme in the citric acid cycle, also known as the Krebs cycle or tricarboxylic acid (TCA) cycle. The TCA cycle is the central metabolic pathway in eukaryotic cells, responsible for generating energy in the form of ATP. IDH3B is the key enzyme that converts the amino acid leucine to the amino acid isoleucine during the TCA cycle.

Mitochondrial disorders are caused by defects in the mitochondria, which are organelles responsible for generating energy for the cell. The most common form of mitochondrial disorders, MELAS, is characterized by a deficiency of IDH3B, leading to a defect in the TCA cycle and a build-up of toxic levels of leucine in the brain. This build-up of leucine can cause a range of symptoms, including progressive muscle weakness, liver disease, and cognitive impairment.

IDH3B as a Drug Target

IDH3B has been identified as a potential drug target for the treatment of mitochondrial disorders due to its role in the TCA cycle and its ability to modulate the levels of toxic leucine in the brain. Several studies have shown that inhibiting IDH3B activity can significantly reduce the build-up of toxic leucine in the brain and improve the symptoms of MELAS.

One of the most promising compounds that has been identified as an IDH3B inhibitor is MK-8628, a drug that was developed by Merck & Co. IDH3B is a key target for MK-8628, and studies have shown that it is effective in animal models of MELAS. In addition, MK-8628 has been shown to be safe and has been approved for clinical trials for the treatment of MELAS.

IDH3B as a Biomarker

In addition to its potential as a drug target, IDH3B has also been identified as a potential biomarker for the diagnosis and prognosis of mitochondrial disorders. The build-up of toxic leucine in the brain is a hallmark of MELAS, and IDH3B has been shown to be a reliable biomarker for the detection of this build-up.

Studies have shown that IDH3B levels are significantly increased in the brain of individuals with MELAS, and that levels decrease significantly in individuals with the condition. Additionally, studies have shown that IDH3B levels can be accurately measured in brain tissue, providing a potential source of biomarker status for the diagnosis and prognosis of MELAS.

Conclusion

In conclusion, IDH3B is a promising drug target for the treatment of mitochondrial disorders due to its role in the TCA cycle and its ability to modulate the levels of toxic leucine in the brain. Studies have shown that IDH3B inhibitors, such as MK-8628, are effective in animal models of MELAS, and IDH3B has been identified as a reliable biomarker for the diagnosis and prognosis of MELAS. Further research is needed to

Protein Name: Isocitrate Dehydrogenase (NAD(+)) 3 Non-catalytic Subunit Beta

Functions: Plays a structural role to facilitate the assembly and ensure the full activity of the enzyme catalyzing the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers

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