The role of auxiliary dihydropyridine receptor subunits in muscle

The skeletal muscle dihydropyridine receptor is a slowly-activating calcium channel that functions as the voltage sensor in excitation-contraction coupling. In addition to the pore-forming α_1S subunit it contains the transmembrane α₂δ-1 and γ₁ subunits and the cytoplasmic β_1a subunit. Although the roles of the auxiliary subunits in calcium channel function have been intensively studied in heterologous expression systems, their functions in excitation-contraction coupling has only recently been elucidated in muscle cells of various null-mutant animal models. In this article we will briefly outline the current state of these investigations. * To whom correspondence should be addressed: Tel.: +43-512-507-3787; Fax: +43-512-507-2836; E-mail: bernhard.e.flucher@uibk.ac.at     

HOW TO DIFFERENTIATE BETWEEN T-CELL-RICH B-CELL LYMPHOMA AND LYMPHOCYTE-PREDOMINANT HODGKIN'S DISEASE. EVIDENCE FOR THE VALUE OF MB1 AND 4KB5 IMMUNOSTAINING

Striking morphological similarities exist between T-cell-rich B-cell lymphoma and lymphocyte-predominant Hodgkin's disease (Hodgkin's paragranuloma), making the distinction between them extremely difficult. Immunohistochemistry provides a means of overcoming this difficulty. Immunostaining with UCHL1, L26, MB1, and 4KB5 was performed on five T-cell-rich B-cell lymphomas and 11 Hodgkin's paragranulomas (7/11 nodular, 4/11 diffuse). L26 stained the tumour cells not only of T-cell-rich B-cell lymphomas, but also of L+H Hodgkin's disease. In contrast, MB1 as well as 4KB5 identified all of the neoplastic cells in 3/5 T-cell-rich B-cell lymphomas, but did not react with the L+H cells in 8/11 Hodgkin's paragranulomas. Some overlap of staining patterns became apparent in the remaining cases, with 2/5 T-cell-rich B-cell lymphomas showing the MB1⁺/4KB5⁺ phenotype in a tumour cell subset only. Similarly, in 3/11 Hodgkin's paragranulomas, some MB1/4KB5-positive L+H cells occurred in addition to MB1/4KB5-negative L+H cells. These cases, nevertheless, could be distinguished from one another by the numbers of MB1/4KB5-positive background lymphocytes, which were scanty or absent in T-cell-rich B-cell lymphomas and more numerous in Hodgkin's paragranulomas.     

Comparing Cause-Specific Infant Mortality in a West German State and the United States of America

ABSTRACT. The infant mortality rate in North Rhine Westphalia (NRW), the most populous West German state, has continuously been around 10 % higher than the German national average in the post-war period. Using white singleton data from the US 1980 National Infant Mortality Surveillance project (NIMS) and similar 1980/1981 data from NRW we compared infant mortality by birthweight and cause to describe the distribution of excess mortality in NRW. The US infant mortality rate was 8.7 deaths per 1000 live births, compared with 13.1/1000 for NRW (rate difference: 4.3/1000). Of the 4.3/1000 overall rate difference, 1.9/1000 was attributable to neonatal deaths, 2.4/1000 to postneonatal deaths. A major proportion, 2.0/1000, of the overall rate difference of 4.3/1000 was attributable to normal birthweight deaths postneonatally. 0.85/1000 of this 2.0/1000 rate difference was attributable to SIDS, 0.44/1000 to external causes and 0.42/1000 to infections.     

Glycogen Storage Disease Type I and III and Pyruvate Carboxylase Deficiency: Results of Long-Term Treatment with Uncooked Cornstarch

ABSTRACT. Three patients with glycogen storage disease type I (GSD-I), three with glycogen storage disease type III (GSD-III) and one with pyruvate carboxylase deficiency (PCD) could be successfully switched over from continuous nocturnal gastric drip feeding (GDF) to nocturnal feeding with uncooked cornstarch in yoghurt or "quark" (CSF) at the age of 4–20 years. The new kind of therapy is much more convenient for the patients. When followed up to 30 months, patients on CSF showed the same clinical and laboratory findings as during the last two years with GDF. CSF was not introduced to three patients with GSD-I. Two of them refused the permanent starch-yoghurt meals. In the third patient the morning blood glucose concentrations were too variable.