PKA-mediated phosphorylation of the regulatory (R) domain plays a major role in the activation of the human cystic fibrosis transmembrane conductance regulator (hCFTR). In contrast, the effect of PKC-mediated phosphorylation is controversial, smaller than that of PKA, and dependent on the cell type. In the present study, we expressed Xenopus CFTR (XCFTR) and hCFTR in Xenopus oocytes and examine...

PKC (protein kinase C) has been known for many years to modulate regulated exocytosis in a wide variety of cell types. In neurons and neuroendocrine cells, PKC regulates several different stages of the exocytotic process, suggesting that these multiple actions of PKC are mediated by phosphorylation of distinct protein targets. In recent years, a variety of exocytotic proteins have been identifi...

Fbw7 (F-box WD40 protein 7) is a major tumour suppressor, which mediates the degradation of several potent oncogenes. PKC (protein kinase C) comprises a serine/threonine kinase family that can promote transformation when dysregulated. In the present study, we investigated the relationship between Fbw7 and PKC. Multiple members of the PKC superfamily interact with the substrate-binding domain of...

Gac Méd Méx Vol. 140, Suplemento No. 3, 2004 Rh haemolytic disease of the fetus and newborn (HDN) is a condition in which the lifespan of the infant’s red cells is shortened by the binding of specific IgG Rh antibodies produced by the mother, which transfer across the placenta. The disease begins in intra-uterine life and shows a wide spectrum of severity. Not all D-positive infants born to mot...

Paroxysmal kinesigenic dyskinesia (PKD) (MIM 128200) is a rare paroxysmal movement disorder that occurs at an estimated prevalence of 1:150,000 individuals. Onset is most commonly in childhood or adolescence, with sporadic and familial cases being reported. PKD is characterized by short and frequent episodes of dystonic or choreiform movements that are precipitated by sudden voluntary movements...

BACKGROUND Alternating hemiplegia of childhood (AHC) is a rare neurological disorder that manifests recurrent attacks of hemiplegia, oculogyric, and choreoathetotic involuntary movements. De novo mutations in ATP1A3 cause three types of neurological diseases: AHC; rapid-onset dystonia-Parkinsonism (RDP); and cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss ...

Twenty-two patients with Parkinson's disease were treated for the periods of up to six months with L-dopa. In nine of the male patients metabolic observations were made after oral administration of (14)C-L-dopa.Peak serum levels of total radioactivity represented small fractions of the dose given and occurred at one to two hours after ingestion. Two-thirds of the dose was excreted as metabolite...

Cadherin adhesion molecules are key determinants of morphogenesis and tissue architecture. Nevertheless, the molecular mechanisms responsible for the morphogenetic contributions of cadherins remain poorly understood in vivo. Besides supporting cell-cell adhesion, cadherins can affect a wide range of cellular functions that include activation of cell signalling pathways, regulation of the cytosk...

It is now generally accepted that Huntington's chorea is inherited through a single dominant autosomal gene but apart from this little is known of the aetiology. Earlier investigators had implicated endocrine factors (Mulon and Porak, 1912; Bize, 1934) but without very convincing evidence. With the advent ofnew drugs that had profound effects on the nervous system the idea of a neuronal basis f...

PKC (protein kinase C) isoenzymes are related protein kinases, involved in many signalling events in normal state and in disease. Basic research into identifying the molecular basis of PKC selectivity led to simple strategies to identify selective competitive inhibitor peptides and allosteric agonist peptides of individual PKC isoenzymes. The strategies and rationale used to identify these pept...