Sex limited ahaptoglobinaemia

Summary Hypohaptoglobinaemia and ahaptoglobinaemia occurred in three generations, mainly to male members of a family. Also small amounts of haptolobin were detected in most of the female relatives. Haemolytic anaemia seemed unlikely and the glucose 6 phosphate dehydrogenase (G.6.P.D.) activity was normal. The probable genotype of these apparently healthy individuals was Hp ²/Hp ². These preliminary data might suggest a defect in control of gene expression by steroid hormones.     

The role of collagenase in emphysema

The extracellular matrix is essential for the integrity of the lung and when disrupted can lead to the architectural changes seen in emphysema. The etiology of emphysema is believed to be due to an imbalance in the proteases and antiproteases within the lung. Studies have focused on elastolytic enzymes as the primary agents in disease pathogenesis, however, recent data suggest that collagenases may also be involved in the destruction of lung tissue in emphysema. It is hoped that this expanded understanding of the pathophysiology of emphysema will lead to improved therapy in the treatment of the disease.     

Immunocytochemical characterization of a new marker of fibrous and reactive astrocytes

A specific monoclonal antiserum (Mab 6.17) inducing a strong immunostaining of the neuromuscular junction has been used to detect the possible occurrence of the corresponding antigen throughout the intact or lesioned central nervous system of adult rats. In intact animals, 6.17-immunolabeling was essentially detected in astrocyte-like structures located in white matter fasciculi of the brain, such as the optic tract, corpus callosum, fornix, and in the white matter of the spinal cord. The astroglial nature of such 6.17-immunolabeled profiles was verified by performing double or triple immunofluorescent labeling with Mab 6.17 and with specific antisera against astrocytic markers, such as S100 protein, glial fibrillary acidic protein and vimentin. In the white matter, all the structures reactive to Mab 6.17 were also reactive to antibodies against S100 protein, glial fibrillary acidic protein and vimentin. On the other hand, astrocytes of the grey matter that were immunoreactive to S100 and glial fibrillary acidic protein but negative to vimentin, were devoid of 6.17-immunoreactivity. After lesions including stab wound through the diencephalon or transection of the spinal cord, a marked increase of 6.17-immunostaining was noted in the regions surrounding the lesions. In these regions, 6.17-immunolabeling was associated with S100-, GFAP- and vimentin-positive astrocytes constituting the glial scar. The ultrastructural localization of 6.17-immunoreactivity indicated that, similar to glial fibrillary acidic protein and vimentin, the recognized antigen was mainly associated with gliofilaments. These observations indicate that, in the central nervous system of adult rats, Mab 6.17 recognizes a molecule associated with gliofilaments, which is essentially associated to reactive astrocytes expressing high levels of vimentin. Received: 2 May 1995 / Accepted: 31 July 1995