JIMD Reports publishes case and short research reports in the area of inherited metabolic disorders. Case reports highlight some unusual or previously unrecorded feature relevant to the disorder, or serve as an important reminder of clinical or biochemical features of a Mendelian disorder.

JIMD Reports publishes case and short research reports in the area of inherited metabolic disorders. Case reports highlight some unusual or previously unrecorded feature relevant to the disorder, or serve as an important reminder of clinical or biochemical features of a Mendelian disorder.

Hepatic Encephalopathy and Nitrogen Metabolism is an interdisciplinary symposium bringing together basic science and clinical applications. It contains up-to-date research findings at the highest scientific level.

This is the first comprehensive volume on Dipeptidyl Aminopeptidases that can be marketed to a wide variety of disciplines, as well as to a variety of clinicians. Leading experts in the field contribute to this state-of-the-art view on these enzymes. This book comes at a time when our understanding of their function is growing ever more rapidly and therapeutic options have become imminent.

The metabolic syndrome is a common syndrome affecting about 20 % of the adult population in Europe, and probably the prevalence is of the same magnitude in other industrialised countries worldwide. It is mainly caused by western lifestyle resulting in abdominal obesity, but also a genetic predisposition plays a role. This syndrome, which is linked to leisure lifestyle and overeating/obesity, can develop into type 2 diabetes, cardiovascular disease and cancer. Therefore, proper treatment and prevention are heavily needed. This book discusses lifestyle intervention and treatment, specifically with pharmacological compounds, in order to formulate a strategy for treating the metabolic syndrome in the daily clinic.

JIMD Reports publishes case and short research reports in the area of inherited metabolic disorders. Case reports highlight some unusual or previously unrecorded feature relevant to the disorder, or serve as an important reminder of clinical or biochemical features of a Mendelian disorder.

Kernicterus (bilirubin encephalopathy) is a highly interesting example of metabolic encephalopathy. It fills all the characteristics of a metabolic encephalopathy in that it can develop rapidly, produces signature signs and symptoms, and is amenable to successful treatment. In the absence of treatment kernicterus can produce devastating sequelae and death. The present volume will examine the biochemistry and physiology of bilirubin as well as its hepatic metabolism and renal excretion. Chapters will elaborate bodily disposition of bilirubin and its neuropathology. Both early treatments and current therapy will be discussed in detail. Phototherapy will be presented, and its efficacy and influence on incidence thoroughly examined.

Excess of homocysteine, a product of the metabolism of the essential amino acid methionine, is associated with poor health, is linked to heart and brain diseases in general human populations, and accelerates mortality in heart disease patients. Neurological and cardiovascular abnormalities occur in patients with severe genetic hyperhomocysteinemia and lead to premature death due to vascular complications. Although it is considered a non-protein amino acid, studies over the past dozen years have discovered mechanisms by which homocysteine becomes a component of proteins. Homocysteine-containing proteins lose their normal biological function and become auto-immunogenic and pro-thrombotic. In this book, the author, a pioneer and a leading contributor to the field, describes up-to date studies of the biological chemistry of homocysteine-containing proteins, as well as pathological consequences and clinical implications of their formation. This is a comprehensive account of the broad range of basic science and medical implications of homocysteine-containing proteins for health and disease.

JIMD Reports publishes case and short research reports in the area of inherited metabolic disorders. Case reports highlight some unusual or previously unrecorded feature relevant to the disorder or serve as an important reminder of clinical or biochemical features of a Mendelian disorder.

JIMD Reports publishes case and short research reports in the area of inherited metabolic disorders. Case reports highlight some unusual or previously unrecorded feature relevant to the disorder, or serve as an important reminder of clinical or biochemical features of a Mendelian disorder.

JIMD Reports publishes case and short research reports in the area of inherited metabolic disorders. Case reports highlight some unusual or previously unrecorded feature relevant to the disorder, or serve as an important reminder of clinical or biochemical features of a Mendelian disorder.

Recent developments in research on diabetes mellitus embrace basic disciplines such as physiology, biochemistry, morphology and evolving sub-specialities. Fundamental studies on the heterogeneous diabetic syndrome are oriented towards regulatory principles of cell metabolism, genetic control mechanisms of insulin biosynthesis and secretion, as well as autoimmune events implicated in selective destruction of pancreatic ss-cell. In parallel, efforts are made for a better understanding of normal versus impaired biological actions, both of insulin and insulin-like growth factor I, and the translocation process of glucose transporters in insulin-responsive target cells."

The scientific advances in the physiology and pathophysiology of adipose tissue over the last two decades have been considerable. Today, the cellular and molecular mechanisms of adipogenesis are well known. In addition, adipose tissue is now recognized as a real endocrine organ that produces hormones such as the leptin acting to regulate food intake and energy balance in the central nervous system, a finding that has completely revolutionized the paradigm of energy homeostasis. Other adipokines have now been described and these molecules are taking on increasing importance in physiology and pathophysiology. Moreover, numerous works have shown that in obesity, but also in cases of lipodystophy, adipose tissue was the site of a local low-grade inflammation that involves immune cells such as macrophages and certain populations of lymphocytes. This new information is an important step in the pathophysiology of both obesity and related metabolic and cardiovascular complications. Finally, it is a unique and original work focusing on adipose tissue, covering biology and pathology by investigating aspects of molecular and cellular biology, general, metabolic, genetic and genomic biochemistry.

JIMD Reports publishes case and short research reports in the area of inherited metabolic disorders. Case reports highlight some unusual or previously unrecorded feature relevant to the disorder or serve as an important reminder of clinical or biochemical features of a Mendelian disorder.

The Fourteenth Midwest Conference on Endocrinology and Metab olism, held at the University of Missouri - Columbia on September 28th and 29th, 1978, brought together several prominent researchers who are authorities on various aspects of the renin-angiotensin system. Each speaker presented an in-depth coverage of a topic related to his own area of expertise, including recent findings from his own research laboratory. Following each presentation thet:e was a general discussion of the material by the speaker and the audience. These presentations and the ensuing discussions are summarized in these published Proceedings. Traditionally the Midwest Conferences on Endocrinology and Metabolism have emphasized breadth as well as depth of coverage of the selected topic; the present Conference is no exception. Perusal of the titles of the presentations will reveal that the Conference dea1th with many different facets of the renin-angiotensin system, including the biochemistry, anatomy, physiology, and comparative endocrinology of this hormonal system, plus special areas of con sideration such as angiotensin receptors, angiotensin-converting enzyme, the control of renin release, angiotensin and aldosterone secretion, and the role of the renin-angiotensin system in the central nervous system. The selection of the renin-angiotensin system as the topic for the present conference was very timely because of the many noteworthy advances in this area in recent years, many by the participants in the Conference. The Editors are very appreciative of the excellent manuscripts which the speakers provided for these Proceedings.

The renewal of interest in excitatory amino acids in the 1980s has altered the direction of neurobiology. L-Glutamate, widely regarded as a general perpetu- ator of nonspecific excitation, has become generally regarded as a widely dis- tributed and important neurotransmitter in the CNS. In the past three decades evidence has accumulated that excitatory amino acids are involved in many neurological diseases and that pharmacological intervention may offer pros- pects of novel and more effective therapies. Numerous studies on experimen- tal animals demonstrate that excitatory amino acid antagonists have anxio- lytic, antiepileptic and muscle relaxant effects. Particular interest has developed in the possible neuroprotective efficacy of excitatory amino acid receptor antagonists in neurological diseases such as hypoxia/ischemia, hypo- glycemia, epilepsy, and chronic neurodegenerative disorders (Huntington's, Alzheimer's, and Parkinson's disease, amyotrophic lateral sclerosis, and AIDS). Thus, the drugs changing neurotransmission mediated by excitatory amino acids possess potential therapeutic value. This volume is part of the Schering Research Foundation Workshop Series. It is the goal of the Schering Research Foundation to promote the asso- ciation between industrial and basic research. The support and the organiza- tion of highly qualified symposia belong to the armamentarium by which this purpose is fulfilled, and we are convinced that the workshop "Excitatory Amino Acids and Second Messenger Systems" has contributed to this. Gunter Stock Ursula-F.

Stjarne: The present review puts the emphasis on two important developments: the discovery that individual postganglionic sympathetic neurons may secrete multiple transmitter substances from different classes of vesicles and by different mechanisms, and the introduction of new techniques which may permit for the first time direct impulse by impulse analysis of transmitter secretion in individual sympathetic nerve varicosities. Illes: Exogenous and endogen opioid peptides elicit a number of effects in the organism, usually by modifying the function of transmitter and hormone systems, for example, activation of multiple opioid receptors. The effects of opioids on transmitter and hormone release have frequently been reviewed. This review gives a detailed overview on the involvement of multiple opioid receptors in these processes. Rothstein: This article gives an overview of the exchange in acidification and transepithelial salt and water transfer in nonepithelial cells. Delineating the exchanger's most important, common features, and concentrating particularly on its role in cell pH and volume regulation.

Systems Metabolic Engineering is changing the way microbial cell factories are designed and optimized for industrial production. Integrating systems biology and biotechnology with new concepts from synthetic biology enables the global analysis and engineering of microorganisms and bioprocesses at super efficiency and versatility otherwise not accessible. Without doubt, systems metabolic engineering is a major driver towards bio-based production of chemicals, materials and fuels from renewables and thus one of the core technologies of global green growth. In this book, Christoph Wittmann and Sang-Yup Lee have assembled the world leaders on systems metabolic engineering and cover the full story - from genomes and networks via discovery and design to industrial implementation practises. This book is a comprehensive resource for students and researchers from academia and industry interested in systems metabolic engineering. It provides us with the fundaments to targeted engineering of microbial cells for sustainable bio-production and stimulates those who are interested to enter this exiting research field.

These two volumes contain articles presented at the Vlth International Symposium on Human Purine and Pyrimidine Metabolism held in Hakone, Japan, July 17 trough 21, 1988. The first meeting of this series of symposia convened in Tel Aviv, Israel, and since then meetings have taken place every three years in various parts of the world. The second meeting was held in Baden, Austria, the third in Madrid, Spain, the fourth in Maastricht, the Netherlands, and fifth in San Diego, California. The Vlth meeting in Hakone marked the first such symposium held in Asia. On occasion of publishing these books, I would like to describe how research in this field has evolved in Japan. Early in the 1950s, I was engaged in clinical practice treating various rheumatic diseases as an orthopedicist, and found that a substantial percentage of our patients had symptoms apparently compatible with gout. During the 1960s, the number of these gouty patients increased, and in the 1970s, research on the pathogenesis of gout was performed on the basis of approximately 2,000 cases of this disease, together with precise epidemiological studies concerning gouty and hyperuricemic individuals. Data derived from the two kinds of study had greatly changed the notion that gout was a rare disease among Japanese. My clinical studies have been succeeded by research at the molecular level on various purine metabolic abnormalities, including not only gout but also other diseases with various symptoms of wide clinical spectra.

JIMD Reports publishes case and short research reports in the area of inherited metabolic disorders. Case reports highlight some unusual or previously unrecorded feature relevant to the disorder, or serve as an important reminder of clinical or biochemical features of a Mendelian disorder.

JIMD Reports publishes case and short research reports in the area of inherited metabolic disorders. Case reports highlight some unusual or previously unrecorded feature relevant to the disorder, or serve as an important reminder of clinical or biochemical features of a Mendelian disorder.

Obesity is a risk factor for breast cancer in older women. A number of adipose-derived and obesity-related factors have been shown to affect tumour cell growth. These include adipokines, insulin, IGF-1 and oestrogens. The majority of obesity-related postmenopausal breast cancers are oestrogen-dependent. Since the ovaries no longer produce oestrogens after menopause, and that circulating levels are negligible, it is evident that it is the oestrogens produced locally within the breast adipose that are responsible for the increased growth of breast cancer cells. Aromatase is the enzyme that converts androgens into oestrogens and its regulation is dependent on the activity of a number of tissue-specific promoters. Targeting oestrogen biosynthesis in obesity may be useful for the prevention of breast cancer. Aromatase inhibitors are efficacious at treating postmenopausal breast cancer and recent studies suggest that they may also be useful in the prevention setting. However, these compounds inhibit the catalytic activity of aromatase and as a consequence lead to a number of undesirable side-effects, including arthralgia and possible cognitive defects due to inhibition of aromatase in the bone and brain, respectively. Novel therapies, such as those employed to treat obesity-associated disease, including anti-diabetics, may prove successful at inhibiting aromatase specifically within the breast. This SpringerBrief will explore all of these issues in depth and the authors are in a unique position to write about this topic, having extensive experience in the field of aromatase research.

Galenical pharmacy or galenics is the science dealing with the pro duction of drug substances from raw materials, the purity of such substances, their formulation into pharmaceutical preparations with the desired effects and safety in use, and the quality control, stability and storage of the preparations. The field has taken its name from the Greek physician Galen (131-201 A.D.), who had a profound influence on medicine for many centuries because he collected and systematized the medicinal knowledge of his time. The discovery of insulin is attributed to Banting and Best who, in 1921, prepared an extract of the pancreas of the fetal calf and showed that the extract was capable of reducing the blood sugar level of a diabetic dog. This outstanding discovery gave rise to the rapid develop ment of the manufacture of insulin of bovine and porcine origin. By 1925, two Danish manufacturers of insulin preparations were established; both have since been in the forefront ofthe development of insulin preparations, the latest achievement being the marketing of human insulin by Novo in 1982. The development of highly purified human insulin produced semisynthetically from porcine insulin or by DNA recombinant methods are significant contributions to safe and efficient insulin therapy. Insulin is a protein which is destroyed in the gastrointestinal tract."