TPK1

TPK1

Target · Metabolic enzyme

TPK1 Thiamin pyrophosphokinase 1 · Thiamine pyrophosphokinase 1

TPK1 (thiamin pyrophosphokinase 1, also known as HTPK1 and PP20) is an enzyme that phosphorylates thiamine to produce thiamine pyrophosphate (TPP). In doing so it links TPP biosynthesis to pyrimidine metabolism. Browse Assay Genie's current range of TPK1 research reagents below, or read on for the biology behind the target.

Homodimeric enzymeThiamine to TPP conversionLinked to thiamine metabolism syndrome
243 aa
Protein length
~27 kDa
Molecular weight
7q35
Gene locus
EC 2.7.6.-
Enzyme class
Target information

What is TPK1 (thiamin pyrophosphokinase 1)?

TPK1 catalyses the phosphorylation of thiamine to thiamine pyrophosphate (TPP), using UTP as the phosphate donor. This ties the biosynthesis of TPP to pyrimidine metabolism. Although the enzyme can also phosphorylate thiamine using ATP and CTP in vitro, it does so with significantly lower efficiency, and there is no evidence that this is physiologically relevant.

TPP is a cofactor of the mitochondrial pyruvate dehydrogenase. By producing it, TPK1 indirectly regulates pyruvate oxidation and lipogenesis. The NCBI Gene summary also describes TPP as a cofactor for some enzymes of the glycolytic and energy production pathways, which places TPK1 in core energy metabolism.

The protein belongs to the thiamine pyrophosphokinase family and functions as a homodimer. The human TPK1 gene is also known by the aliases HTPK1 and PP20, and the mouse ortholog is Tpk1. Defects in the TPK1 gene are a cause of thiamine metabolism dysfunction syndrome-5, which makes the enzyme relevant to studies of inherited disorders of thiamine metabolism.

Thiamine metabolism

TPK1 converts thiamine to the active cofactor thiamine pyrophosphate, making it a central enzyme for studying how cells generate TPP.

Energy metabolism

TPP is a cofactor for enzymes of glycolytic and energy production pathways, so TPK1 activity connects to pyruvate oxidation and cellular energy supply.

Inherited metabolic disease

Defects in the TPK1 gene cause thiamine metabolism dysfunction syndrome-5, making it a focus for research into thiamine-related disorders.

Pyrimidine and lipid metabolism

Because TPK1 uses UTP and influences pyruvate dehydrogenase activity, it links pyrimidine metabolism with pyruvate oxidation and lipogenesis.

Background

TPK1 frequently asked questions

What does TPK1 do?

TPK1 catalyses the phosphorylation of thiamine to thiamine pyrophosphate (TPP), using UTP. TPP is a cofactor of the mitochondrial pyruvate dehydrogenase, so TPK1 indirectly regulates pyruvate oxidation and lipogenesis. It also links TPP biosynthesis to pyrimidine metabolism.

What other names is TPK1 known by?

The HGNC full name is thiamin pyrophosphokinase 1, and UniProt lists the protein as thiamine pyrophosphokinase 1. Aliases include HTPK1 and PP20. The enzyme carries the EC number 2.7.6.- and belongs to the thiamine pyrophosphokinase family.

Is TPK1 linked to disease?

Yes. According to the NCBI Gene summary, defects in the TPK1 gene are a cause of thiamine metabolism dysfunction syndrome-5. A mouse ortholog, Tpk1, exists; check the datasheet of any product for its validated reactivity.

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Related targets

Other thiamine-metabolism enzymes, pyruvate dehydrogenase components and thiamine transporters with products at Assay Genie.

Gene and protein annotations from HGNC, NCBI Gene and UniProt Q9H3S4; product specifications from Assay Genie datasheets.

TPK1 Product listing