Galactosemia: the good, the bad, and the unknown

Alpha-D-galactose is metabolized in species ranging from E. coli to mammals predominantly via a series of sequential reactions collectively known as the Leloir pathway. Deficiency of any one of these enzymes in humans results in a form of the inherited metabolic disorder, galactosemia, although the symptoms and severity depend upon the enzyme impaired, and the degree of functional deficiency (Tyfield and Walter, 2002, The Metabolic and Molecular Bases of Inherited Disease. New York: McGraw Hill.). Studies of these enzymes, and the disorders associated with their loss, have led to a much deeper appreciation of the intricate and interwoven levels of regulation that govern their normal function. These insights have further identified likely mediators of outcome severity in patients, and have enabled a rational approach to the development of novel strategies of intervention.     

Superantigens: the good, the bad, and the ugly

Increasing evidence suggests that superantigens play a role in immune-mediated diseases. Superantigens are potent activators of CD4+ T cells, causing rapid and massive proliferation of cells and cytokine production. This characteristic of superantigens can be exploited in diseases where strong immunologic responses are required, such as in the B16F10 animal model of melanoma. Superantigen administration is able to significantly enhance ineffective anti-tumor immune responses, resulting in potent and long-lived protective anti-tumor immunity. However, superantigens are more well-known for the role they play in diseases. Studies using an animal model for neurologic demyelinating diseases such as multiple sclerosis show that superantigens can induce severe relapses and activate autoreactive T cells not involved in the initial bout of disease. This may also involve epitope spreading of disease. Superantigens have also been implicated in acute diseases such as food poisoning and TSS, and in chronic diseases such as psoriasis and rheumatoid arthritis. Viral superantigens are also involved in the disease process, including superantigens derived from human immunodeficiency virus and mouse mammary tumor virus. Finally, immunotherapies that ameliorate the role played by superantigens in disease are discussed.     

Galen: On Blood,the Pulse,and the Arteries

This essay examines several important issues regarding Galen’s depiction of the physiology of the arteries. In the process some of Galen’s supporting doctrines on the blood and pulse will also be discussed in the context of a coherent scientific explanation. It will be the contention of this essay that though Galen may often have a polemical goal in mind, he correctly identifies the important and complex role of the arteries in human biological systems.     

Baculoviruses and apoptosis: the good, the bad, and the ugly

Since 1991, when a baculovirus was first shown to inhibit apoptosis of its host insect cells, considerable contributions to our knowledge of apoptosis have arisen from the study of these viruses and the anti-apoptotic genes they encode. Baculovirus anti-apoptotic genes include p35, which encodes the most broadly acting caspase inhibitor protein known, and iap (inhibitor of apoptosis) genes, which were the first members of an evolutionarily conserved gene family involved in regulation of apoptosis and cytokinesis in organisms ranging from yeast to humans. Baculoviruses also provide an ideal system to study the effects of an apoptotic response on viral pathogenesis in an animal host. In this review, I discuss a number of interesting recent developments in the areas of apoptotic regulation by baculoviruses and the effects of apoptosis on baculovirus replication and pathogenesis.     

Death at the zoo: the media,science, and reality

Media characterizations of zoo and aquarium animal deaths were randomly monitored on the internet for a 20‐month period (September 2003–May 2005). Based on 148 samples collected, it was possible to classify articles into one of four categories, which were operationally defined: 1) dispassionate observers; 2) accusers; 3) sympathizers; and 4) balancers. In addition, with the notable exception of seven cases, all of the articles examined focused on large, charismatic mammals, such as gorillas, dolphins, lions, and elephants. Although a majority of the articles examined (70.4%) were either dispassionate and objective or sympathetic, nearly a third (29.6%) were either accusatory or attempted to balance the accusatory statements of animal rights activists with sympathetic statements from zoo professionals. Recommendations are offered for how zoos should deal with the increasing media and public interest in zoo animal deaths, including: 1) a greater commitment to studying the reasons for mortality in a wide variety of species; and 2) an increased investment in record keeping and analysis, which should allow zoos to calculate average life spans in animal populations and to monitor and assess the risk of certain lethal diseases on a real‐time basis. Comparisons are drawn between zoo veterinary practices and human medicine, which are both inexact sciences. Suggestions are made for how the public and key decision‐makers can distinguish between media reports on zoo animal deaths that are legitimate cause for concern vs. those that are sensationalist and meant to generate controversy and sell papers. A greater focus on the science of zoo animal death is necessary for accredited zoos to maintain the public's confidence in their animal care practices. Zoo Biol 0:1–15, 2006. © 2006 Wiley‐Liss, Inc.