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Titlebook: Disorders of Porphyrin Metabolism; Michael R. Moore,Kenneth E. L. McColl,Abraham Gold Book 1987 Springer Science+Business Media New York 1

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发表于 2025-3-21 19:09:43 | 显示全部楼层 |阅读模式
书目名称Disorders of Porphyrin Metabolism
编辑Michael R. Moore,Kenneth E. L. McColl,Abraham Gold
视频video
丛书名称Topics in Hematology
图书封面Titlebook: Disorders of Porphyrin Metabolism;  Michael R. Moore,Kenneth E. L. McColl,Abraham Gold Book 1987 Springer Science+Business Media New York 1
描述Each of the four authors of this book has a particular interest in disorders of porphyrin metabolism and special experience in their management. Their individual involvement in the field varies from 12 to 52 years and, combined, represents more than a century of personal experience. Since it has been written by both basic scientists and practicing physicians, the book is intended to be of value to all those involved in porphyrin metab­ olism and the porphyrias. It is hoped that the fascination of porphyrin metabolism and the clinical challenge of the porphyrias experienced by each of the authors will be conveyed to the readers. Michael R. Moore Kenneth E. L. McColl Claude Rimington Abraham Goldberg vii CONTENTS Color Plates ............................................ xvii 1. The History, Classification, and Incidence of the Porphyrias 1 1.1. History ........................................ 1 1.1.1. Early Chemistry . . . . . . . . . . . . . . . . . . . . . . . . . . . 2 1.1.2. Early Descriptions of Porphyria .............. 4 1.1.3. Biochemical Developments .................. 4 1.1.4. Acute Porphyria . . . . . . . . . . . . . . . . . . . . . . . . . . . 7 1.1.5. A Complete Pathway .
出版日期Book 1987
关键词chemistry; classification; development; management; metabolism
版次1
doihttps://doi.org/10.1007/978-1-4684-1277-2
isbn_softcover978-1-4684-1279-6
isbn_ebook978-1-4684-1277-2
copyrightSpringer Science+Business Media New York 1987
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发表于 2025-3-21 20:51:36 | 显示全部楼层
Porphyrins and Enzymes of the Heme Biosynthetic Pathway,idges (Fig. 7). This is a rigid planar structure onto which eight side chains can be attached at positions 1 to 8 in the Fischer nomenclature. The type of side chain determines the physical characteristics of the porphyrin. The four pyrrole rings are designated A, B, C, and D and the four methine br
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Drugs, Chemicals, and Porphyria,h century. This produced the first example of acute porphyria (Stokvis, 1889) and was followed by a number of similar cases. Since that time, a number of commonly used drugs have been linked with the development of acute attacks of these diseases. Workers have used the ability of certain compounds t
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Cutaneous Hepatic Porphyria,ks of neurovisceral dysfunction. The basic biochemical defect is reduced activity of the enzyme uroporphyrinogen decarboxylase (URO.D) in the liver, which results in overproduction and increased excretion of uroporphyrin and other polycarboxylated porphyrins. CHP differs from the other porphyrias in
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Erythropoietic Protoporphyria,arly identified by Magnus .. (1961) and Langhöf .. (1961), although there had been earlier reports (Kosenow and Treibs, 1953). At this stage, it was named erythropoietic protoporphyria (EPP), but it became clear from the investigations of Gray .. (1964) and Barnes .. (1968) and from the studies of m
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Congenital Porphyria, of photosensitivity in early life. There is overproduction of porphyries of the series 1 variety. These accumulate in the normoblasts of the bone marrow and are excreted in excess in the urine and feces. Porphyries are also deposited in the bones and teeth, resulting in a pink-brown discoloration.
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Porphyrins in Diseases Other than the Porphyrias,ias. Studies of porphyrin metabolism in these conditions provide a useful insight into the underlying disease process as well as being of clinical value in both diagnosing and assessing the severity of the disorder. The abnormal porphyrin metabolism identified in tumor cells has been employed as an
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