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 The Pathophysiology of Sickle Cell Disease. Cellular, Molecular and Biochemical Mechanisms
Наименование:

The Pathophysiology of Sickle Cell Disease. Cellular, Molecular and Biochemical Mechanisms

автор Katherine W. издательство Книга по Требованию Медико-биологические дисциплины
Cтраниц 192        мягкая обложка       
Аннотация:It is less well known as a disease that targets the microvasculature, characterized by increased expression of cell adhesion molecules, enhanced production of reactive oxygen species, abnormal blood cell-endothelial cell interactions in postcapillary venules and altered vasomotor responses in arterioles. This work considers sickle cell disease vascular pathology in terms of the cellular, molecular and biochemical players that participate in the evolution,progression and resolution of vasoocclusive crises. These microvascular responses are crucial to the initiation and progression of vasoocclusive crises, the disease’s critical pathophysiological event that leads to pain crises, organ injury and early mortality. The work provides a comprehensive overview of the complex pathophysiology of sickle cell disease and is an ideal reference source for professionals in the medical and scientific community. Sickle cell disease is best known as an inherited hemolytic disorder of defective hemoglobin that distorts red blood cell morphology, function and lifespan.
Автор: Katherine W.
Издательство: Книга по Требованию
Год:2009
Цена:
2768 руб
Нет в наличии
Поиск: The Pathophysiology of Sickle Cell Disease. Cellular, Molecular and Biochemical Mechanisms
НОВЫЙ ГОД СИМВОЛ 2013 ГОДАУКРАШЕНИЯ
КАРНАВАЛ НА НОВЫЙ ГОД РАСПРОДАЖА
Ортомолекулярная медицина Orthomol Устрание причин заболеваний. Ортомоль это новый метод для лечения и профилактики болезней. Orthomol - ортомолекулярная медицина.
Лизосомы

Нерасщепленный субстрат такой гидролазы накапливается в лизосомах, что и обусловливает патологию. Обычно такие болезни вызываются мутацией в структурном гене, кодирующем отдельную гидролазу. Наиболее тяжелые симптомы характеризуют редкую форму патологии, называемую I-клеточной болезнью (i clusio cell disease). У таких больных в лизосомах фибробластов отсутствуют почти все гидролитические ферменты, а соответствующие нерасщепленные субстраты накапливаются в клетках в виде крупных «включений. I-клеточная болезнь обусловлена рецессивной мутацией единственного гена. Это означает, что она проявляется только у людей, получивших дефектные копии гена от обоих родителей. При изучении I-клеточной болезни оказалось, что в лизосомах все гидролазы отсутствуют, но в крови они обнаруживаются. Из этого следует, что структурные гены, кодирующие их, не повреждены. Аномалия в данном случае вызвана нарушениями процесса сортировки в аппарате Гольджи, в результате чего гидролазы вместо того, чтобы поступать в лизосомы, секретируются.
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