Lessons from Laron Syndrome (LS) 1966-1992: A Model of GH and IGF-1 Action and Interaction : Ares-Serono Symposium, Lisbon, May 25-26, 1992Zvi Laron, John S. Parks Karger Medical and Scientific Publishers, 01.01.1993 - 367 Seiten Laron Syndrome (LS) is a disease characterized by resistance to growth hormone (GH) and caused by molecular defects of the GH receptor, leading to a deficiency of insulin-like growth factor-1 (IGF-1) and a significant impairment of growth. The syndrome is a unique human model of a peptide hormone receptor defect and GH-IGF-1 interaction. It offers the opportunity to study the consequences of long-term IGF-1 deficiency and the role of IGF-1 in growth and metabolism. With the recent biosynthesis of IGF-1 and the initiation of IGF-1 therapy, a whole new world of basic biological interactions is opening up. This volume brings together leading researchers in genetics, endocrinology, pediatrics, biochemistry, physiology and pharmacology to summarize what is now known about LS, and to discuss the lessons learned from studying and treating this disease since its initial description in 1966. Special features include the demography of LS, and the first data on the long-term IGF-1 treatment of a large group of patients. |
Inhalt
First Direct Evidence | 24 |
Laron Z Tel Aviv Cognitive | 53 |
An Additional Patient | 70 |
Laron Syndrome A Review of 8 Iranian Patients with | 93 |
The First Reported Case | 101 |
Tchelet A Sakal E Vogel T Rehovot Krivi G G Creely D St Louis Mo | 114 |
Defects of the GH Receptor in Laron Syndrome | 127 |
A Stanford Calif GuevaraAguirre J Quito Rosenbloom A L | 140 |
LongTerm IGF1 Treatment of Children | 226 |
IGF1 Treatment of Adult | 237 |
Nishi Y Hiroshima Okada S Tajiri H Nose O Osaka Hasegawa Y | 244 |
Prévot C Rappaport R Paris Effects of Nutrient | 254 |
Serum Prolactin | 261 |
Animal Models of Abnormal GH Receptor | 282 |
M Leuven TixierBoichard M JouyenJosas Vleurick L Hever | 288 |
Hull K L Harvey S Edmonton Marsh J A Ithaca N Y Boone F McCann | 294 |
Insulin Growth Factor Binding Proteins in Laron Syndrome | 167 |
Interest of IGF1 and IGFBPS | 181 |
Ontogeny | 192 |
The Role of IGF1 in Ovarian Physiology Basic | 202 |
Eshet R Klinger B Tel Aviv Werner H Roberts C T Jr LeRoith D | 209 |
TCell Receptor Activated Lymphocytes Secrete IFG | 216 |
M Darras V M Leuven TixierBoichard M JouyenJosas Van | 310 |
Comparison between hGH and IGF1 | 319 |
Are There Direct NonIGF1Mediated Effects | 338 |
Divergence | 346 |
Summary and Conclusions | 359 |
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Acad Sci USA adults antibody basal Basel biochemical blood glucose cDNA Clin Endocrinol Metab clinical concentrations decrease defect deletion dwarf chickens dwarfism with high exon genetic GH binding protein GH deficiency GH receptor gene GH-BP activity GH-R GHBP GHRD growth hormone binding growth hormone deficiency growth hormone receptor height hormone binding protein hormone receptor deficiency human growth hormone hypoglycemia IGF-1 levels IGF-1 receptor IGF-1 treatment IGF-I IGFBP-3 IGHD increase insulin receptor insulin-like growth factor Karger Klinger Laron dwarfism Laron dwarfs Laron Syndrome Laron type Laron-type dwarfism Lessons from Laron liver LS patients Met-hGH metabolic molecular mRNA mutations Natl Acad Sci ng/ml normal Parks JS eds patients with Laron patients with LS Pediatr Adolesc Endocrinol Pertzelan plasma Proc Natl Acad puberty pygmies sequence serum GH-BP serum IGF-1 sex-linked sex-linked dwarf somatomedin splice studies Syndrome LS tissue type dwarfism µg/l
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