Multifocal ectopic premature Purkinje-related complexes syndrome is a rare form of arrhythmia involving the entire His-Purkinje system (HPS), characterized by frequent polymorphic ventricular contractions (PVCs) relatively narrow QRS, which could be arising from HPS and associated with high probability dilated cardiomyopathy. We presented clinical courses 6 pediatric patients multifocal syndrom...

We investigated the subthreshold properties of an erg (ether-à-go-go-related gene) K(+) current in Purkinje cells of neonatal mice. Action potentials recorded from Purkinje cells in cerebellar slices exhibited a decreased threshold potential and increased frequency of spontaneous and repetitive activity following application of the specific erg channel blocker E-4031. Accommodation was absent b...

Studies of Purkinje cell dendritic spine proliferation after transplantation of cytosine arabinoside (Ara C) treated organotypic cerebellar cultures with glia and granule cells, either separately and in combination, were reviewed. Exposure of cerebellar explants to Ara C for the first 5 days in vitro results in the destruction of granule cells, the only excitatory cortical neurons, and oligoden...

The thyroid hormone l-3,3',5-triiodothyronine (T3) plays an important role during cerebellar development. Perinatal T3 deficiency leads to severe cellular perturbations, among them a striking reduction in the growth and branching of Purkinje cell dendritic arborization. The molecular mechanisms underlying these effects are poorly understood. Despite the well documented broad expression of thyro...

Canine hearts were isolated 6 hours, 24 hours, 3 days, 6 days, and 4 weeks after coronary artery ligation and were perfused with blood from support dogs. Electrograms were recorded from Purkinje fibers in the infarcted zone and from Purkinje fibers and ventricular muscle in noninfarcted areas. Administration of 2.5 mEq of KC1 into the coronary circulation of the infarcted hearts abolished nonin...

We present a Purkinje-myocardium electrophysiology model that includes the scar plus a detailed Purkinje network, and compare simulated activation times to those obtained by electro-anatomical mapping in vivo. The results illustrate the importance of using sufficiently dense Purkinje networks in patient-specific studies.

This article reminisces about the life and key scientific achievements of Jan Evangelista Purkinje (1787-1869), a versatile 19th century Czech pioneer of modern experimental physiology. In 1804, after completing senior high school, Purkinje joined the Piarist monk order, but, after a 3-yr novitiate, he gave up the religious calling "to deal more freely with science." In 1818, he earned a Medica...

The highly specific connection patterns of the mature CNS are shaped through finely regulated processes of axon growth and retraction. To investigate the relative contribution of cell-autonomous mechanisms and extrinsic cues in these events, we examined the development of Purkinje axon intracortical plexus in the rat cerebellum. During the first postnatal week, several new processes sprout from...

To investigate whether the c-fms proto-oncogene plays a role in the CNS, we examined its expression in mouse brain. We found that c-fms-positive Purkinje cells first appeared in caudal cerebellum at postnatal day 0 (P0) arranged in a parasagittal manner, and most Purkinje cells gradually became positive by P6. This differential expression was not seen from P7 to adulthood, and the parasagittal ...

Early postnatal developmental changes in N-methyl-d-aspartate (NMDA) receptor (NR) subunits regulate cerebellar granule cell maturation and potentially Purkinje cell development. We therefore investigated Purkinje cell morphology in slice cultures from mice with genetic subunit exchange from NR2C to NR2B (NR2C-2B). NR2C-2B Purkinje cells after 12 days in vitro showed a significantly impaired de...