Cilia and cilia-related disorders

Abstract

Extract

Among the puzzling aspects of the recently described autosomal recessive polycystic kidney disease (ARPKD) of sheep (Johnstone et al 2005 Johnstone, AC, Davidson, BI, Roe, AR, Eccles, MR and Jolly, RD. 2005. Congenital polycystic kidney disease in lambs. New Zealand Veterinary Journal, 53: 307–314. [Taylor &; Francis Online], [Web of Science ®] , [Google Scholar]) is the multiplicity of organ systems affected and the phenotypic variations that occur between the various animal species. It has been shown in studies of other inherited polycystic kidney disease syndromes that the normal gene products of a mutant are likely to be involved in ciliary structure and function. The studies that have led to this recognition can be expected to eventually provide the basis for a better understanding of the functioning of this organelle, and the pathogenesis of lesions in the related diseases.

It is well known that cilia are important in the perception of light, olfactory stimuli and sound and that motile cilia provide cell motility (e.g. sperm) and transport of mucus and other fluids. Recent research has indicated that the involvement of the primary cili-um/basal body complex is of central importance in the detection and cellular response to extracellular movement of fluid, critical phases of embryonic development, cell cycle regulation and maintenance of cell polarity.

Confocal microscopic studies of renal cyst epithelium in ovine ARPKD have shown that only 30% of cells have a cilium and that these are often truncated (McGlashan et al 2005 McGlashan, SR, Poole, CA, Stayner, C, Johnstone, AC, Eccles, MR and Jensen, CG. 2005. “Primary cilia in fibrosis associated with two models of polycystic kidney disease”. In Proceedings of the 45th Annual Conference of the American Society for Cell Biology December 10–14 [Google Scholar]). The observation, although preliminary, strongly supports the argument that the mutant gene in this disease of sheep normally encodes for a protein essential for primary ciliary function.     

Impacts of rising atmospheric carbon dioxide on model terrestrial ecosystems

In model terrestrial ecosystems maintained for three plant generations at elevated concentrations of atmospheric carbon dioxide, increases in photosynthetically fixed carbon were allocated below ground, raising concentrations of dissolved organic carbon in soil. These effects were then transmitted up the decomposer food chain. Soil microbial biomass was unaffected, but the composition of soil fungal species changed, with increases in rates of cellulose decomposition. There were also changes in the abundance and species composition of Collembola, fungal-feeding arthropods. These results have implications for long-term feedback processes in soil ecosystems that are subject to rising global atmospheric carbon dioxide concentrations.     

Electrical conductivity in the precambrian lithosphere of western canada

The subcrustal lithosphere underlying the southern Archean Churchill Province (ACP) in western Canada is at least one order of magnitude more electrically conductive than the lithosphere beneath adjacent Paleoproterozoic crust. The measured electrical properties of the lithosphere underlying most of the Paleoproterozoic crust can be explained by the conductivity of olivine. Mantle xenolith and geological mapping evidence indicate that the lithosphere beneath the southern ACP was substantially modified as a result of being trapped between two nearly synchronous Paleoproterozoic subduction zones. Tectonically induced metasomatism thus may have enhanced the subcrustal lithosphere conductivity of the southern ACP.     

Congenital polycystic kidney disease in lambs

AIM: To describe the pathology and inheritance of a congenital polycystic kidney disease (PKD) of sheep.

METHODS: Mode of inheritance of PKD was investigated by evaluation of results of the disorder from planned matings in two consecutive years within subsets of a flock that had a high prevalence of PKD in lambs. Gross pathological and histopath- ological studies were based on tissues derived from this study. Haematoxylin and eosin (H&E)-stained paraffin sections of kidney, liver, extrahepatic biliary and pancreatic ducts, pancreas and epididymis were used to describe the lesions.

RESULTS: Twenty-five lambs affected by PKD, of both sexes, were born, numbers in accord with those expected for an autosomal recessive disorder in the population studied. In all cases for which tissues were available, the renal, bile ductal (intrahepatic and extrahepatic), pancreatic and epididymal tissues had widespread dysplastic changes and associated cyst formation.

CONCLUSIONS: The findings of renal cysts in conjunction with cysts in other organs are unifying features in many of the human and animal forms of PKD and suggest a related pathogenic and genetic base consistent with an autosomal recessive disorder.     

Structural basis of transcription: separation of RNA from DNA by RNA polymerase II

The structure of an RNA polymerase II-transcribing complex has been determined in the posttranslocation state, with a vacancy at the growing end of the RNA-DNA hybrid helix. At the opposite end of the hybrid helix, the RNA separates from the template DNA. This separation of nucleic acid strands is brought about by interaction with a set of proteins loops in a strand/loop network. Formation of the network must occur in the transition from abortive initiation to promoter escape.     

Administration of Thyroxine Affects the Morphometric Parameters and VEGF Expression in the Uterus and Placenta and the Uterine Vascularization but does Not Affect Reproductive Parameters in Gilts During Early Gestation

The aim of this study was to evaluate the effects of thyroxine administration on morphometric parameters, expression of vascular endothelial growth factor (VEGF) and vascularization in the uterus and placenta and reproductive parameters in gilts at 70 days of gestation. At 150 days of age, i.e., before first heat, 20 gilts were randomly divided into two experimental groups: treated (n = 10) and control (n = 10). The treated group received a daily dose of 400 μg of l ‐thyroxine (T₄) in their diet until slaughter and the control group received only typical meals. Before artificial insemination, blood was collected to determine plasma total T₄. The gilts were inseminated in the second oestrus and slaughtered at 70 days of gestation. The foetal thyroid follicular epithelium height, number, size and weight of foetuses; foetal myogenesis, corpora lutea number, embryonic mortality rate, uterine weight, placental weight and placental fluid volume were measured. Histomorphometric and immunohistochemical analysis of uterus and placenta were determined. The averages of all variables were compared by the Student’s t‐test. The gilts treated with thyroxine showed significant increase of plasma total T₄. At 70 days of gestation, the heights of the trophoblastic epithelium, endometrial epithelium and endometrial gland epithelium were significantly higher in the group treated with T₄. The expression of cytoplasmatic and nuclear VEGF in trophoblastic cells and the number of blood vessels per field in endometrial stroma were significantly higher in the gilts treated with T₄. No other significant differences between groups were obtained with respect to other parameters (p > 0.05). We conclude that oral administration of T₄ up to 70 days of pregnancy in gilts affects the morphometric parameters, the expression of placental VEGF and the uterine vascularization but does not affect reproductive parameters in gilts during early gestation.