Serine hydroxymethyltransferase, mitochondrial

Definition:

Catalyzes the cleavage of serine to glycine accompanied with the production of 5,10-methylenetetrahydrofolate, an essential intermediate for purine biosynthesis. Serine provides the major source of folate one-carbon in cells by catalyzing the transfer of one carbon from serine to tetrahydrofolate. Contributes to the de novo mitochondrial thymidylate biosynthesis pathway via its role in glycine and tetrahydrofolate metabolism: thymidylate biosynthesis is required to prevent uracil accumulation in mtDNA. Also required for mitochondrial translation by producing 5,10-methylenetetrahydrofolate; 5,10-methylenetetrahydrofolate providing methyl donors to produce the taurinomethyluridine base at the wobble position of some mitochondrial tRNAs. Associates with mitochondrial DNA. In addition to its role in mitochondria, also plays a role in the deubiquitination of target proteins as component of the BRISC complex: required for IFNAR1 deubiquitination by the BRISC complex.

References:

[1]. Daniel F Bogenhagen, et al. The layered structure of human mitochondrial DNA nucleoids. J Biol Chem. 2008 Feb 8;283(6):3665-3675. [Content Brief]

[2]. Hui Zheng, et al. A BRISC-SHMT complex deubiquitinates IFNAR1 and regulates interferon responses. Cell Rep. 2013 Oct 17;5(1):180-93. [Content Brief]

[3]. Raphael J Morscher, et al. Mitochondrial translation requires folate-dependent tRNA methylation. Nature. 2018 Feb 1;554(7690):128-132. [Content Brief]

[4]. Donald D Anderson, et al. Identification of a de novo thymidylate biosynthesis pathway in mammalian mitochondria. Proc Natl Acad Sci U S A. 2011 Sep 13;108(37):15163-8. [Content Brief]

[5]. Denise R Minton, et al. Serine Catabolism by SHMT2 Is Required for Proper Mitochondrial Translation Initiation and Maintenance of Formylmethionyl-tRNAs. Mol Cell. 2018 Feb 15;69(4):610-621.e5. [Content Brief]

[6]. Giorgio Giardina, et al. How pyridoxal 5'-phosphate differentially regulates human cytosolic and mitochondrial serine hydroxymethyltransferase oligomeric state. FEBS J. 2015 Apr;282(7):1225-41. [Content Brief]

[7]. Àngels García-Cazorla, et al. Impairment of the mitochondrial one-carbon metabolism enzyme SHMT2 causes a novel brain and heart developmental syndrome. Acta Neuropathol. 2020 Dec;140(6):971-975. [Content Brief]

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