標(biāo)題: Titlebook: Lysosomal Storage Disorders; John A. Barranger,Mario A. Cabrera-Salazar Book 2007 Springer-Verlag US 2007 Glycogen.Lipid.Polysaccharid.enz [打印本頁] 作者: CANTO 時(shí)間: 2025-3-21 19:40
書目名稱Lysosomal Storage Disorders影響因子(影響力)
作者: chiropractor 時(shí)間: 2025-3-21 23:25
Book 2007. In no other group of disorders has knowledge and clinical utility progressed so speedily. Recall that the organelle was described in just 1955. Since then, the biochemical alterations and storage materials were described, the enzyme deficiencies discovered, the gene coding of these glycoproteins c作者: Serenity 時(shí)間: 2025-3-22 04:02 作者: FIG 時(shí)間: 2025-3-22 04:49
From Lysosomes to Storage Diseases and Back: A Personal Reminiscence,tell that could perhaps be of interest, and hold some instructive aspects, especially to the younger generations. In order to stay within the boundaries of my competence, my story is restricted to events with which I have been personally associated. To put those events作者: 休息 時(shí)間: 2025-3-22 10:23 作者: 有抱負(fù)者 時(shí)間: 2025-3-22 13:20
John A. Barranger,Mario A. Cabrera-SalazarDescribes the nature of the lysosomal dysfunction and diseases as well as potential future treatments and therapies.Provides the historical evolution of the field.Organized as a textbook with disease-作者: 寬度 時(shí)間: 2025-3-22 17:12
http://image.papertrans.cn/l/image/589248.jpg作者: 一個(gè)攪動(dòng)不安 時(shí)間: 2025-3-22 23:47 作者: Asparagus 時(shí)間: 2025-3-23 01:52
978-1-4419-4366-8Springer-Verlag US 2007作者: 有害 時(shí)間: 2025-3-23 06:02
From Lysosomes to Storage Diseases and Back: A Personal Reminiscence,t has enjoyed so many recent developments of major importance, including singularly successful clinical applications, unusual for genetic diseases. The task, I felt, would be much better fulfilled by one of the pioneers of the field, several of whom are still in the forefront of research today. Then作者: Affiliation 時(shí)間: 2025-3-23 11:33
Lysosomal Biogenesis and Disease,but more recently developed from molecular studies on vesicular traffic and the cell biology of specific endosomal–lysosomal proteins. The dynamics of the endomembrane system is discussed and includes the concepts of biosynthesis, vesicular traffic, protein processing, secretion, enzyme uptake, and 作者: heterogeneous 時(shí)間: 2025-3-23 17:48 作者: GRIEF 時(shí)間: 2025-3-23 19:48
Retroviral Vectors for Gene Therapy,sed on murine leukemia virus (MLV) and related retroviruses have been employed in almost half of the current gene therapy clinical protocols (.). The major advantages of retroviral vectors are (1) ease of manipulation for insertion of the therapeutic gene; (2) ability to stably integrate into the ta作者: 碎石 時(shí)間: 2025-3-24 01:54 作者: 填滿 時(shí)間: 2025-3-24 03:41 作者: JEER 時(shí)間: 2025-3-24 10:26
Adeno-Associated Viral-Mediated Gene Therapy of Lysosomal Storage Disorders,nzymes that are responsible for the catabolism of a variety of macromolecules. The LSD as a group, present with an incidence of approximately 1 in 7500 live births making it one of the more prevalent groups of genetic diseases. The description of the pathophysiology of LSD, as well as their biochemi作者: 逢迎白雪 時(shí)間: 2025-3-24 11:58 作者: Ordnance 時(shí)間: 2025-3-24 18:40 作者: Myosin 時(shí)間: 2025-3-24 21:28
Substrate Reduction Therapy,h, 2002). The majority are autosomal recessive disorders and result from mutations in the genes that encode the catabolic enzymes of the lysosome (Winchester, 2004). Clinically they are highly variable (Beck, 2001) but typically have a neurodegenerative course and commonly present in infancy or earl作者: 里程碑 時(shí)間: 2025-3-25 01:45
Newborn Screening for Lysosomal Storage Disorders,2 in the Massachusetts Department of Public Health and was based on the work of Dr. Robert Guthrie for the detection of phenylketonuria. The initiation of screening for phenylketonuria and its subsequent expansion to include congenital hypothyroidism, galactosemia, congenital adrenal hyperplasia, bi作者: 金桌活畫面 時(shí)間: 2025-3-25 05:11 作者: 機(jī)警 時(shí)間: 2025-3-25 11:06
The GM1 Gangliosidoses,body. They manifest a predominantly neurological phenotype, a fact that is probably related to their high prevalence in nervous tissues. The typical presentation is that of a progressive neurodegenerative disease with onset in early life followed by loss of acquired developmental milestones, dementi作者: 過份艷麗 時(shí)間: 2025-3-25 11:49 作者: Tailor 時(shí)間: 2025-3-25 18:38
,Acid Sphingomyelinase-Deficient Niemann–Pick Disease, numerous reports of infants with similar clinical manifestations appeared, and in 1927 Ludwig Pick distinguished this disorder from infantile Gaucher disease based on the differential appearance of the bone marrow foam cells (Pick, 1927). The first adults with this disease were described in 1946 (P作者: indigenous 時(shí)間: 2025-3-25 20:48
Krabbe Disease (Globoid Cell Leukodystrophy),atural activator/ protective protein, saposin A. The latter was first established in a mouse model, but the first human GLD patient due to saposin A deficiency has very recently been discovered. The primary natural substrate of the enzyme, galactosylceramide, is nearly exclusively localized in the m作者: 鉆孔 時(shí)間: 2025-3-26 03:18 作者: 可轉(zhuǎn)變 時(shí)間: 2025-3-26 07:27 作者: aplomb 時(shí)間: 2025-3-26 10:44 作者: Sputum 時(shí)間: 2025-3-26 15:29
Doug Brooks,Emma Parkinson-Lawrencere professionals spend thousands of hours studying the science and technology of healthcare, and years or even decades putting into practice recent findings in molecular biology, clinical diagnostics, and therapeutics. By contrast, the topic of leadership and the traits of effective leaders tend to 作者: 乳白光 時(shí)間: 2025-3-26 18:52
Roscoe O. Bradyionals spend thousands of hours studying the science and technology of healthcare, and years or even decades putting into practice recent findings in molecular biology, clinical diagnostics, and therapeutics. By contrast, the topic of leadership and the traits of effective leaders tend to receive re作者: 撤退 時(shí)間: 2025-3-26 21:20
Roscoe O. Brady M.D.,Roscoe O. Brady Jr. M.D., Ph.D.ionals spend thousands of hours studying the science and technology of healthcare, and years or even decades putting into practice recent findings in molecular biology, clinical diagnostics, and therapeutics. By contrast, the topic of leadership and the traits of effective leaders tend to receive re作者: Statins 時(shí)間: 2025-3-27 03:59
Seon-Hee Kim PhD,Paul D. Robbinsre professionals spend thousands of hours studying the science and technology of healthcare, and years or even decades putting into practice recent findings in molecular biology, clinical diagnostics, and therapeutics. By contrast, the topic of leadership and the traits of effective leaders tend to 作者: 無王時(shí)期, 時(shí)間: 2025-3-27 06:35
Angela Montecalvo,Andrea Gambotto,Leonardo D’Aiutore professionals spend thousands of hours studying the science and technology of healthcare, and years or even decades putting into practice recent findings in molecular biology, clinical diagnostics, and therapeutics. By contrast, the topic of leadership and the traits of effective leaders tend to 作者: 矛盾心理 時(shí)間: 2025-3-27 12:37
Dolan Sondhi,Neil R. Hackett,Stephen M. Kaminksy,Ronald G. Crystalionals spend thousands of hours studying the science and technology of healthcare, and years or even decades putting into practice recent findings in molecular biology, clinical diagnostics, and therapeutics. By contrast, the topic of leadership and the traits of effective leaders tend to receive re作者: Gudgeon 時(shí)間: 2025-3-27 14:53
Mario A. Cabrera-Salazar MD,Seng H. Chengionals spend thousands of hours studying the science and technology of healthcare, and years or even decades putting into practice recent findings in molecular biology, clinical diagnostics, and therapeutics. By contrast, the topic of leadership and the traits of effective leaders tend to receive re作者: 模范 時(shí)間: 2025-3-27 20:27 作者: Blazon 時(shí)間: 2025-3-28 00:48
Alessandra Biffi,Luigi Naldiniship competencies from ‘best practice’ health organizations .This innovative book analyses the evolving nature of leadership, exploring an ever-increasing range of theoretical concepts and applying these to practices within healthcare organisations. A wide range of theories are covered, from behavio作者: 值得尊敬 時(shí)間: 2025-3-28 02:59 作者: CLASP 時(shí)間: 2025-3-28 07:19
C. Ronald Scott,Frantisek Turecek,Michael H. Gelbions scholars tend to either ignore or disregard the role of individual leaders is, on the whole, true.. Undeniably, contemplating a role for the personality and abilities of any particular leader is difficult to reconcile with aspirations to scientific rigor. In one of the rare articles in the inte作者: Spongy-Bone 時(shí)間: 2025-3-28 11:08 作者: hankering 時(shí)間: 2025-3-28 15:33
Gustavo Charria-Ortizes leadership within an inter-cultural society.This book examines the concept of leadership from within the Islamic worldview, exploring its meaning and various manifestations through textual evidence from the two primary sources of Islam, The Qur’an and hadith. Using this theoretical framework conc作者: 補(bǔ)充 時(shí)間: 2025-3-28 22:47
Gustavo A. Charria-Ortiz MDvement disorders specialist at The Parkinson’s Institute, Sunnyvale, California, and the Institute of Neurology, Queen Square, London. He is on the editorial board for several international journals. He has published over 700 publications, including more than 535 peer-reviewed international papers. 作者: 愛社交 時(shí)間: 2025-3-28 23:26 作者: PHONE 時(shí)間: 2025-3-29 06:37 作者: 盡責(zé) 時(shí)間: 2025-3-29 07:56 作者: Hirsutism 時(shí)間: 2025-3-29 12:18 作者: 自制 時(shí)間: 2025-3-29 19:27 作者: 搖曳 時(shí)間: 2025-3-29 20:45
Roscoe O. Brady M.D.,Roscoe O. Brady Jr. M.D., Ph.D.mposed of insightful and thought-provoking essays on the key facets of leadership, this book is designed to meet the needs of several important constituencies, including educators of health professionals who wish to incorporate leadership into their educational programs; health professional organiza作者: Adrenal-Glands 時(shí)間: 2025-3-30 00:58 作者: 瘋狂 時(shí)間: 2025-3-30 06:33
Angela Montecalvo,Andrea Gambotto,Leonardo D’Aiutovely....Composed of insightful and thought-provoking essays on the key facets of leadership, this book is designed to meet the needs of several important constituencies, including educators of health professionals who wish to incorporate leadership into their educational programs; health professional organiza978-1-84800-942-4978-1-84800-943-1作者: compassion 時(shí)間: 2025-3-30 11:30 作者: pester 時(shí)間: 2025-3-30 16:12 作者: JOT 時(shí)間: 2025-3-30 17:01 作者: esoteric 時(shí)間: 2025-3-30 22:45 作者: 東西 時(shí)間: 2025-3-31 01:06 作者: 拱形大橋 時(shí)間: 2025-3-31 05:27 作者: ADJ 時(shí)間: 2025-3-31 10:14
Gustavo A. Charria-Ortiz MDfor Nijmegen in 2012. Since 2017, he has served on the Executive Scientific Advisory Board of The Michael J Fox Foundation. In 2018, he was elected as member of the Royal Holland Society of Sciences and Humanities. In 2018, he won the Tom Isaacs award as recognition of his longstanding achievements 作者: 轎車 時(shí)間: 2025-3-31 15:37
Volkmar Gieselmanncademic professionals for a life as active citizens in a democratic society and the development of the professionals’ personality is elaborated and discussed. In closing, it is argued that in spite of changing concepts in nursing curriculum, the objective of higher education of nurses throughout the作者: 膽汁 時(shí)間: 2025-3-31 20:17 作者: 未開化 時(shí)間: 2025-3-31 21:58 作者: 防水 時(shí)間: 2025-4-1 03:09
Lysosomal Biogenesis and Disease,–lysosome biogenesis and function is susceptible to the effects of mutation and can therefore result in a genetic disease. Even for defects in the same gene, different mutations may have dramatically different effects based on how the message and gene product interact with the processing machinery a作者: 一窩小鳥 時(shí)間: 2025-4-1 09:03
The Concept of Treatment in Lysosomal Storage Diseases,ved that this cerebroside accumulated in the organs and tissues of patients with Gaucher disease. However, the optical rotation of an aqueous solution of the sugar derived from the accumulating cerebroside was incompatible with its being galactose. In 1934, Aghion reported that the sugar moiety of t作者: Haphazard 時(shí)間: 2025-4-1 10:55
