標(biāo)題: Titlebook: Inborn Errors of Metabolism in Humans; Monograph based upon Forrester Cockburn,Richard Gitzelmann Conference proceedings 1982 The Society f [打印本頁(yè)] 作者: 投射技術(shù) 時(shí)間: 2025-3-21 17:40
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書(shū)目名稱(chēng)Inborn Errors of Metabolism in Humans讀者反饋學(xué)科排名
作者: nascent 時(shí)間: 2025-3-21 22:53
Recent studies on the maturation of lysosomal enzymesly, that the location and history of the hydrolases affects their structure. Because acid hydrolases are glycoproteins, the structural variations may be in the polypeptide or the carbohydrate portions of the molecule.作者: 繁重 時(shí)間: 2025-3-22 00:56
Artificial cell-encapsulated enzymes and adsorbents in congenital metabolic disordersh extracellular or intracellular accumulation of metabolites, which might be helped by the removal of the metabolites from the extracellular fluid; and (2) those with intracellular accumulation of metabolites which cannot be removed by extracellular means.作者: calorie 時(shí)間: 2025-3-22 06:29
Murine mutations affecting myelination: models to study myelin diseases in the humanthe peripheral nervous system (PNS). During the process of myelin formation, the cytoplasm is extruded and the cellular leaflets are fused to form the major dense line (Figure 13.2). The external surfaces come together and form the myelin intraperiod line (also called minor dense line, Figure 13.2).作者: Ischemic-Stroke 時(shí)間: 2025-3-22 10:42 作者: 完整 時(shí)間: 2025-3-22 16:22
Homocystinuria: clinical and biochemical heterogeneitynotypes, both clinical and biochemical in nature, are often caused by a variety of different genotypes; in other words, heterogeneity abounds. One such disorder demonstrating heterogeneity is the condition first described as homocystinuria.作者: Ebct207 時(shí)間: 2025-3-22 18:27 作者: atopic-rhinitis 時(shí)間: 2025-3-23 00:00 作者: 矛盾心理 時(shí)間: 2025-3-23 05:09
Molecular aspects of genetic heterogeneity that it is a common and widespread feature of inherited disease.. In other words, one can now say that ‘genetic heterogeneity’ is the rule rather than the exception, and its possible implications must be considered in the analysis of any inherited clinical disorder.作者: 釋放 時(shí)間: 2025-3-23 06:31 作者: 壓碎 時(shí)間: 2025-3-23 11:33
Inborn errors of metabolism affecting brain development — Introductionl does not explain completely the abnormal function which is frequently observed in IEM. The difficulty lies in determining the hierarchy of causes, distinguishing between cause and effect and in relating structure and function.作者: Generalize 時(shí)間: 2025-3-23 14:12
N. A. J. Carson regulatory output. So, is independence of competition authorities useful to society in the end? This book will appeal to upper-level students and scholars interested in competition policy, regulatory agencies, and European public policy.?.978-1-349-93392-1978-1-137-58814-2Series ISSN 2524-7263 Series E-ISSN 2524-7271 作者: 傾聽(tīng) 時(shí)間: 2025-3-23 20:53 作者: 嚙齒動(dòng)物 時(shí)間: 2025-3-23 23:59
W. M. Pardridgeng point in its history. In May 2004, ten countries will join a new European Union composed of twenty-five Member States and 450 million people. This massive accession to the EU is of paramount importance to th978-3-642-07358-8978-3-540-24712-8作者: commute 時(shí)間: 2025-3-24 04:39
R. O. Brady,J. A. Barranger,P. G. Pentchev,F. S. Furbish,A. E. Gal作者: comely 時(shí)間: 2025-3-24 09:20
undertake excessive risks to face competition. This explains to a certain extent the existence of some regulatory arrangements – such as the requirement of relatively high minimum capital – that promote bank concentration. But, at the same time, the absence of competition can weaken market disciplin作者: Conscientious 時(shí)間: 2025-3-24 11:37
W. L. Nyhan. Economic analysis has been playing an increasingly significant role in the Commission’s examination of competition law cases. This holds true in particular for merger control. Here, however, the Commission has had to accept some poi- ant defeats in court, such as the Court’s reversals of Airtours-作者: 愛(ài)花花兒憤怒 時(shí)間: 2025-3-24 17:01 作者: Constrain 時(shí)間: 2025-3-24 20:23
policy in the "acquis communautaire". European competition policy is now at a tuming turning point in its history. In May 2004, ten countries will join a new European Union composed of twenty-five Member States and 450 million people. This massive accession to the EU is of paramount importance to th作者: Cerebrovascular 時(shí)間: 2025-3-25 00:19
E. F. Neufeldn terms of the principle of .; and subsequently through its analysis of .. The chapter ends with an assessment of the policy, identifying the criticisms that have been levelled at the Article 86[82] regime, and drawing rather negative conclusions about the effectiveness of the Commission’s monopoly 作者: 預(yù)示 時(shí)間: 2025-3-25 05:04 作者: 消音器 時(shí)間: 2025-3-25 07:33
licy. They find support from economists, who propose an international migration order to control migration and to co-ordinate migration policies. Analogous to the GATT, this proposal is named GAMP, the General Agreement on Migration Policies..The assumption behind the proposal is that unregulated mi作者: reperfusion 時(shí)間: 2025-3-25 12:30 作者: Coeval 時(shí)間: 2025-3-25 18:52
N. Baumann,F. Lachapelle,J.-C. Turpinon does. Rules and their supporting institutions evolve spontaneously because individuals discover that in their voluntary interactions the rules are co-ordinated more effectively under one system or process than under another (Benson, 1989). The relative effectiveness of rules and institutions are 作者: Heresy 時(shí)間: 2025-3-25 20:39 作者: 美食家 時(shí)間: 2025-3-26 03:51
F. A. Hommes,A. G. Eller,E. H. Taylornsure the desirability of these competitive processes, competition among institutions also requires a framework of rules channelling the competitive processes into desirable directions. Before we begin our discussion on these issues, however, some clarifying comments are in order to narrow down what作者: Nonporous 時(shí)間: 2025-3-26 07:22 作者: motivate 時(shí)間: 2025-3-26 11:23
consider it timely to write a new volume, as experimentation and modelling of competition- coexistence relationships have well advanced beyond the milestone books of the 1980s. In addition to this, the global loss of biodiversity has led to a renewed interest in mechanisms that explain spatial diffe作者: 不遵守 時(shí)間: 2025-3-26 15:33 作者: RAGE 時(shí)間: 2025-3-26 20:33
Molecular aspects of genetic heterogeneity the increasing use of the term ‘genetic heterogeneity’. It is usually meant to imply that what appears to be the same clinical syndrome may be the consequence of a number of quite different gene mutations. That this could indeed occur has, of course, been recognized for a long time.. But whereas in作者: 消息靈通 時(shí)間: 2025-3-27 00:11
Inborn errors of purine metabolismrs, each of them individually uncommon, has pointed out the ways in which molecular expression of gene action takes place in man. It has given us new understanding of mechanisms of human variation. It has pioneered in the field of prenatal diagnosis and has permitted new approaches to questions of p作者: LIMN 時(shí)間: 2025-3-27 04:57 作者: agenda 時(shí)間: 2025-3-27 05:32
Homocystinuria: clinical and biochemical heterogeneitynded to the inactivity of a specific enzyme in the intermediary metabolism of a substance. As our knowledge of these hereditary disorders continued to expand, it became obvious that we must modify these earlier views. It is now common experience among the inborn errors of metabolism that similar phe作者: 令人發(fā)膩 時(shí)間: 2025-3-27 09:50
Hereditary defects of steroid biosynthesisr subsequent synthesis also occur in the placenta, in the liver and in other tissues. Several hereditary enzyme defects are known. Many more are theoretically possible, but have not yet been described. Best known are those of the adrenal steroids which cause adrenal insufficiency and the adrenogenit作者: 兩棲動(dòng)物 時(shí)間: 2025-3-27 14:05 作者: 貧困 時(shí)間: 2025-3-27 21:36
Recent studies on the maturation of lysosomal enzymes in body fluids and sometimes in microsomes. Recent work has shown that the fine structure of these hydrolases determines their location, and conversely, that the location and history of the hydrolases affects their structure. Because acid hydrolases are glycoproteins, the structural variations may 作者: Kaleidoscope 時(shí)間: 2025-3-27 22:30
Enzyme substitution by fibroblast transplantationr because an enzyme which controls a single metabolic step is entirely absent or has a greatly reduced activity. More than 330 such disorders have now been described., more than 30 of which are lysosomal storage diseases.作者: 復(fù)習(xí) 時(shí)間: 2025-3-28 02:30 作者: gregarious 時(shí)間: 2025-3-28 08:33 作者: Throttle 時(shí)間: 2025-3-28 14:13
Mutations in mice affecting brain development and their correlations with human diseases multiplication and myelination, outgrowth of dendrites and axons, and establishment of neuronal connections occur. Mice appear to be particu- larly useful for studying hereditary inborn errors of brain metabolism and differentiation. In mice, these processes occur postnatally during a short span of作者: 山羊 時(shí)間: 2025-3-28 16:35
Murine mutations affecting myelination: models to study myelin diseases in the humanmyelin membrane is an extension of the oligodendrocyte plasma membrane in the central nervous system (CNS) and of the Schwann-cell plasma membrane in the peripheral nervous system (PNS). During the process of myelin formation, the cytoplasm is extruded and the cellular leaflets are fused to form the作者: Ligneous 時(shí)間: 2025-3-28 19:25
The effect of phenylalanine on myelin metabolism in adolescent ratsHowever, the phenylalanine-4-mono-oxygenase is not expressed in teratocarcinoma cells (S. Molenaar and F. A. Hommes, unpublished observation), so it will be difficult, although not impossible, to search for the appropriate mutant cell line. Therefore, chemically-induced models have to be used. Prese作者: 表示向前 時(shí)間: 2025-3-29 00:01
terize this sector explain the high regulation of its economic activities. This feature is geared towards solving the important information problems faced by the economic actors, so as to protect savers’ interests and ensure the stability of the financial sector as a whole. This objective, neverthel作者: 有限 時(shí)間: 2025-3-29 06:31 作者: 制定 時(shí)間: 2025-3-29 08:31 作者: yohimbine 時(shí)間: 2025-3-29 15:11
N. A. J. Carsonion enforcement does not have a significant impact on the agThis book investigates the crucial EU policy of competition, which is enforced by the Commission and by national agencies that enjoy various degrees of autonomy from their governments. More and more policy-making activities are nowadays del作者: 夜晚 時(shí)間: 2025-3-29 17:49
A. Prader,M. Zachmannion enforcement does not have a significant impact on the agThis book investigates the crucial EU policy of competition, which is enforced by the Commission and by national agencies that enjoy various degrees of autonomy from their governments. More and more policy-making activities are nowadays del作者: OCTO 時(shí)間: 2025-3-29 23:13
W. M. Pardridgeuse of competition from various experts in the field.With ex1 by Karel Van Miert Since the early years of the European Community, competition policy has played a decisive role in the creation of a more prosperous, competitive and integrated European economy. Over the last forty years, the action of 作者: 移動(dòng) 時(shí)間: 2025-3-30 03:36 作者: Chipmunk 時(shí)間: 2025-3-30 05:00 作者: 漂白 時(shí)間: 2025-3-30 09:14 作者: Apogee 時(shí)間: 2025-3-30 13:34
hts. The United States has experienced a level of legal immigration unseen since the beginning of the century, and the number of illegal immigrants to the US is supposed to have increased as well. Due to liberal asylum laws, asylum-seekers have flooded Western Europe, which has been exacerbated by a作者: 多樣 時(shí)間: 2025-3-30 20:12 作者: TRACE 時(shí)間: 2025-3-30 22:01 作者: 簡(jiǎn)略 時(shí)間: 2025-3-31 02:08 作者: municipality 時(shí)間: 2025-3-31 06:54
F. A. Hommes,A. G. Eller,E. H. Tayloronary approach to the process of competition among institutions. For a more thorough analysis we will draw a parallel between ordinary market competition and the subject of our interest, competition in the realm of institutions. We shall focus, in particular, on two issues. First, the role of the co作者: 開(kāi)始沒(méi)有 時(shí)間: 2025-3-31 12:28 作者: 會(huì)議 時(shí)間: 2025-3-31 14:06
e past century, various theoretical, laboratory and field studies have sought to understand how species that compete can coexist. In the early 1980s, two influential reviews (Schoener 1983; Connell 1983) demonstrated that, at least in published studies, interspecific competition appears to be freque作者: SHOCK 時(shí)間: 2025-3-31 20:11 作者: CODA 時(shí)間: 2025-4-1 00:46
978-94-009-7327-5The Society for the Study of Inborn Errors of Metabolism 1982作者: Adrenaline 時(shí)間: 2025-4-1 03:51
Overview: 978-94-009-7327-5978-94-009-7325-1作者: integrated 時(shí)間: 2025-4-1 06:54
Enzyme substitution by fibroblast transplantationr because an enzyme which controls a single metabolic step is entirely absent or has a greatly reduced activity. More than 330 such disorders have now been described., more than 30 of which are lysosomal storage diseases.
