A recombinant E. coli co-expressing ALA synthase (hemA), NADP-dependent malic enzyme (maeB), and dicarboxylic acid transporter (dctA) was reported to synthesize porphyrin derivatives including iron-containing heme. To enhance the synthesis of bacterial heme, five genes of the porphyrin biosynthetic pathway [pantothenate kinase (coaA), ALA dehydratase (hemB), 1-hydroxymethylbilane synthase (hemC...

Pantothenate kinase (PanK) is a regulatory enzyme that controls coenzyme A (CoA) biosynthesis. The association of PanK with neurodegeneration and diabetes suggests that chemical modifiers of PanK activity may be useful therapeutics. We performed a high throughput screen of >520000 compounds from the St. Jude compound library and identified new potent PanK inhibitors and activators with chemical...

Pantothenate kinase-associated neurodegeneration (PKAN) is a familial or sporadic disease characterized by extrapyramidal and corticospinal signs with dementia. Patients show iron accumulation in the basal ganglia, with neuronal loss and gliosis. A mutation of pantothenate kinase (PANK2) gene localized on chromosome 20p13 has been described in familiar forms, as well as in sporadic patients. We...

Hallervorden and Spatz first described, in a sibship of 12, five sisters with clinically increasing dysarthria and progressive dementia, whose brains showed a brown discoloration of the globus pallidus and substantia nigra. Subsequently the basis has been shown to be a neurodegeneration with brain iron accumulation or pantothenate kinase-associated neurodegeneration due to mutations in the pant...

The inhibition rate of L(-)pantothenate as an antagonist of D(+)pantothenate was discussed from the viewpoint of growth and lipid metabolism. The growth rate of mice given pantothenate-deficient diets containing L(-)pantothenate (300 mg per 100g diet) was markedly decreased, and such pantothenate-deficient symptoms as "spectacle eyes" appeared. However, it was recovered by the simultaneous addi...