Molecular medicine reports

Silencing of pantothenate kinase 2 reduces endothelial cell angiogenesis.

PMID 30221726


Coenzyme A (CoA) is an essential cofactor of cellular metabolism that is involved in ~4% of cellular reactions. Its de novo production relies on five subsequent enzymatic steps, starting with the phosphorylation of vitamin B5. Pantothenate kinase 2 (PANK2) and coenzyme A synthase (COASY) catalyze the first and last steps of this pathway. Mutations in these genes lead to severe and progressive movement disorders, with neurodegeneration and iron accumulation in the basal ganglia, known as PANK2‑ and COASY protein‑associated neurodegeneration, respectively. Given the ubiquitous role of CoA in cellular metabolism, it is still not clear why patients carrying PANK2 and COASY mutations develop almost exclusively neurological symptoms. Important clues are the energetic profile of neural cells as well as the high levels of PANK2 expression in the brain; however, other features may contribute to this selective tissue vulnerability. Notably, when pank2 or coasy expression was suppressed in zebrafish evident perturbation of neuronal development was observed, as well as severe defects in vasculature formation. Supplementation of CoA to fish water prevented the appearance of the phenotype, thereby confirming the specific connection with the availability of the metabolic cofactor. The present study investigated the associations between PANK2 defects and angiogenesis in a mammalian setting, and revealed that PANK2 expression was required for normal angiogenetic properties of human umbilical vein endothelial cells.

Related Materials

Product #



Molecular Formula

Add to Cart

2-Methyl-1,3-thiazole, AldrichCPR