BACKGROUND To clarify the pathogenesis of cerebellar Purkinje cell death in patients with Menkes kinky hair disease (MD), a disorder of copper absorption, we investigated the morphological and functional abnormalities of residual Purkinje cells in MD patients and the mechanism of cell death. METHODS Seven MD patients and 39 neurologically normal autopsy cases were studied. We performed histop...

The adult cerebellar cortex is comprised of reproducible arrays of transverse zones and parasagittal stripes of Purkinje cells. Adult stripes are created through the perinatal rostrocaudal dispersion of embryonic Purkinje cell clusters, triggered by signaling through the Reelin pathway. Reelin is secreted by neurons in the external granular layer and deep cerebellar nuclei and binds to two high...

Introduction: Acrylamide (ACR) consumption is increasing all over the world. There are some evidence on the literature about its neurotoxic effect on mature animals, but the effects of ACR on postnatal development have been less studied. The purpose of this study was to evaluate the effects of ACR on development of cortical layer, white matter, and number of Purkinje cells of the cerebellum in ...

Purkinje cells [5], also called Purkinje neurons, are neurons in vertebrate animals located in the cerebellar cortex of the brain. Purkinje cell bodies are shaped like a flask and have many threadlike extensions called dendrites, which receive impulses from other neurons called granule cells [6]. Each cell also has a single projection called an axon, which transmits impulses to the part of the ...

Purkinje cells [5], also called Purkinje neurons, are neurons in vertebrate animals located in the cerebellar cortex of the brain. Purkinje cell bodies are shaped like a flask and have many threadlike extensions called dendrites, which receive impulses from other neurons called granule cells [6]. Each cell also has a single projection called an axon, which transmits impulses to the part of the ...

Estimates of total number and mean volume of Purkinje cells in five human and nine rat cerebella were obtained using stereological methods based on unbiased principles and estimators. The average total number of Purkinje cells was 30.5 106 (CV = SD/mean = 0.13) in humans and 0.61 106 (CV = 0.21) in rats. Thus the total number of Purkinje cells was 50 times higher in the human compared with rats...

Dendritic spines of Purkinje cells form excitatory synapses with parallel fiber terminals, which are the primary sites for cerebellar synaptic plasticity. Nevertheless, how density and morphology of these spines are properly maintained in mature Purkinje cells is not well understood. Here we show an activity-dependent mechanism that represses excessive spine development in mature Purkinje cells...

The appearance of cyclic guanosine 3':5'-monophosphate-dependent protein kinase (cGK), an enzyme that may be involved in the regulation of various aspects of neuronal function and that is highly concentrated in cerebellar Purkinje cells, was studied in the developing and adult monkey cerebellum by indirect immunofluorescent staining. The appearance and distribution of cGK immunoreactivity were ...

The development of the atrioventricular bundle (AVB) and ventricular Purkinje system and their innervation have been studied in fetal sheep from 27 to 140 days gestation (term is 147 days). The AVB initially consisted of a primordium, which lacked innervation and was composed of small, relatively undifferentiated myocytes. Differentiation of Purkinje-like cells within the AVB began near its dis...

In immunohistochemical reactions against glutamic acid decarboxylase (GAD), gamma-aminobutyric acid (GABA) and glycine (Gly), neurons in the mouse cerebellum showed the following reactivities: (1) the dendrites and cell bodies of the Purkinje cells were only GAD-positive, but their axonal terminals were GABA- and GAD-positive; (2) in both stellate and basket cells, the cell bodies and terminals...