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      Endocrine assessment, molecular characterization and treatment of growth hormone insensitivity disorders.

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          Abstract

          Advances in the diagnosis and treatment of growth hormone insensitivity disorders have occurred in the past 15 years. We discuss the current status of endocrine and molecular evaluation, focusing on the pediatric age range. All the identified mutations of the growth hormone receptor are included. Treatment with recombinant human insulin-like growth factor (rhIGF) 1 in classical cases is summarized and new targets for treatment are discussed, together with therapy using the complex formed between rhIGF1 and rhIGF-binding protein 3.

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          Growth hormone insensitivity associated with a STAT5b mutation.

          Vivian Hwa, Caroline K Buckway, J. Heinrich … (2003)
          Article 0 comments Cited 118 times – based on 0 reviews     Review now
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            Structural design and molecular evolution of a cytokine receptor superfamily.

            J. Bazan (1990)
            A family of cytokine receptors comprising molecules specific for a diverse group of hematopoietic factors and growth hormones has been principally defined by a striking homology of binding domains. This work proposes that the approximately 200-residue binding segment of the canonical cytokine receptor is composed of two discrete folding domains that share a significant sequence and structural resemblance. Analogous motifs are found in tandem approximately 100-amino acid domains in the extracellular segments of a receptor family formed by the interferon-alpha/beta and -gamma receptors and tissue factor, a membrane tether for a coagulation protease. Domains from the receptor supergroup reveal clear evolutionary links to fibronectin type III structures, approximately 90-amino acid modules that are typically found in cell surface molecules with adhesive functions. Predictive structural analysis of the shared receptor and fibronectin domains locates seven beta-strands in conserved regions of the chain; these strands are modeled to fold into antiparallel beta-sandwiches with a topology that is similar to immunoglobulin constant domains. These findings have strong implications for understanding the evolutionary emergence of an important class of regulatory molecules from primitive adhesive modules. In addition, the resulting double-barrel design of the receptors and the spatial clustering of conserved residues suggest a likely binding site for cytokine ligands.
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              Laron syndrome (primary growth hormone resistance or insensitivity): the personal experience 1958-2003.

              Zvi Laron (2004)
              Clinical and laboratory investigations starting in 1958 of a group of dwarfed children resembling isolated GH deficiency but who had very high serum levels of GH led to the description of the syndrome of primary GH resistance or insensitivity (Laron syndrome) and subsequently to the discovery of its molecular defects residing in the GH receptor and leading to an inability of IGF-I generation. With the biosynthesis of IGF-I in 1986, therapeutic trials started. Continuously more and more patients are being diagnosed in many parts of the world with a variety of molecular defects. This syndrome proved to be a unique model that enables the study of the consequences of GH receptor defects, the physiopathology of GH-IGF-I disruption, and comparison of the GH-independent IGF-I effects. This review presents the personal experience gained from the study follow-up and treatment of the 60 patients followed up for many years in the Israeli cohort.
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                Author and article information

                Journal
                Journal ID (iso-abbrev): Nat Clin Pract Endocrinol Metab
                Title: Nature clinical practice. Endocrinology & metabolism
                Publisher: Springer Science and Business Media LLC
                ISSN: 1745-8366
                ISSN (Print): 1745-8366
                Publication date (Electronic): Jul 2006
                Volume: 2
                Issue: 7
                Affiliations
                [1 ] Paediatric Endocrinology Unit, William Harvey Research Institute, St Bartholomew's Hospital and the London School of Medicine & Dentistry, London, UK. m.o.savage@qmul.ac.uk
                Article
                Publisher Item ID: ncpendmet0195
                DOI: 10.1038/ncpendmet0195
                PubMed ID: 16932322
                SO-VID: 77b17150-0424-4dac-8031-3d11882052f3
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