0000000000452220
AUTHOR
James F. Reynolds
Morphological studies in canine (Dalmatian) neuronal ceroid-lipofuscinosis.
Dalmatian dogs may develop a neuronal or generalized ceroid-lipofuscinosis (NCL) which strongly resembles that seen in English setters, especially as to the ultrastructural changes and ubiquity of the stored lipopigments and the retinal pathology, while differing clinically from the disorder of English setters in that the disease has a longer course of up to 5 or 6 yr. Clinical onset is at about age 6 months; however, an unequivocal morphological diagnosis is possible between the 4th and 5th month of life in biopsied skin. Detailed data of additional investigations are in progress and are awaiting later publication. Thus, NCL in the Dalmatian dog, though not yet as thoroughly investigated a…
The clinical spectrum of alpha-L-iduronidase deficiency.
We present five patients with alpha-L-iduronidase deficiency who do not have the typical Hurler or Scheie phenotypes; they are compared to 28 similarly atypical cases from the literature. Phenotypic differences are pointed out and intrafamilial similarities stressed. Among the various possible explanations for this situation, the existence of genetic compounds seems acceptable for some of the cases, but others seem to be caused by different mutations. The elucidation of these alternative possibilities from recent biochemical research is discussed.
Changes in evapotranspiration and phenology as consequences of shrub removal in dry forests of central Argentina
More than half of the dry woodlands (forests and shrublands) of the world are in South America, mainly in Brazil and Argentina, where in the last years intense land use changes have occurred. This study evaluated how the transition from woody-dominated to grass-dominated system affected key ecohydrological variables and biophysical processes over 20 000 ha of dry forest in central Argentina. We used a simplified surface energy balance model together with moderate-resolution imaging spectroradiometer–normalized difference vegetation index data to analyse changes in above primary productivity, phenology, actual evapotranspiration, albedo and land surface temperature for four complete growing …