Copeptin as a biomarker and a diagnostic tool in the evaluation of patients with polyuria–polydipsia and hyponatremia
Publication date: Available online 16 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): M. Christ-Crain, N.G. Morgenthaler, W. Fenske Copeptin is part of the 164 amino acid precursor protein preprovasopressin together with vasopressin and neurophysin II. During precursor processing, copeptin is released together with vasopressin. Copeptin concentrations respond as rapidly as vasopressin to changes in osmolality, a decrease in blood pressure or stress and there is a close correlation of vasopressin and copeptin concentrations. For these reasons, copeptin is pro...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - March 3, 2016 Category: Endocrinology Source Type: research

Renal aquaporins and water balance disorders
Publication date: Available online 2 March 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Hanne B. Møller, Cecilia H. Fuglsang, Robert A. Fenton Aquaporins (AQPs) are a 13 member family (AQP0-12) of proteins that act as channels, through which water and, for some family members, glycerol, urea and other small solutes can be transported. Aquaporins are highly abundant in kidney epithelial cells where they play a critical role with respect to water balance. In this review we summarize the current knowledge with respect to the localization and function of AQPs w...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - March 3, 2016 Category: Endocrinology Source Type: research

A copeptin-based classification of the osmoregulatory defects in the Syndrome of Inappropriate Antidiuresis
Publication date: Available online 2 March 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): W. Fenske, B. Sandner, M. Christ-Crain The syndrome of inappropriate antidiuretic hormone secretion (SIADH), also referred to as syndrome of inappropriate antidiuresis (SIAD), is the most common cause of hyponatremia characterized by extracellular hypotonicity and impaired urine dilution in the absence of any recognizable nonosmotic stimuli for the antidiuretic hormone arginine vasopressin (AVP). Hyponatremia in SIADH is primarily the result of excessive water retention caused ...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - March 3, 2016 Category: Endocrinology Source Type: research

Genetic Forms of Nephrogenic Diabetes Insipidus: Vasopressin Receptor Defect (X-linked) and Aquaporin Defect (Autosomal Recessive and Dominant)
Publication date: Available online 2 March 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Daniel G. Bichet, Detlef Bockenhauer Nephrogenic diabetes insipidus (NDI), which can be inherited or acquired, is characterized by an inability to concentrate urine despite normal or elevated plasma concentrations of the antidiuretic hormone, arginine vasopressin (AVP). Polyuria with hyposthenuria and polydipsia are the cardinal clinical manifestations of the disease. About 90 percent of patients with congenital NDI are males with X-linked NDI who have mutations in the vasopress...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - March 3, 2016 Category: Endocrinology Source Type: research

Histopathology of NET: Current concepts and new developments
Publication date: Available online 26 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Anja M. Schmitt, Annika Blank, Ilaria Marinoni, Paul Komminoth, Aurel Perren The diagnosis of neuroendocrine tumors is based on their histopathologic appearance and immunohistochemical profile. With the WHO 2010 classification formal staging and grading was introduced for gastro-entero-pancreatic NET, however, the nomenclature for lung neuroendocrine tumors still relies on the carcinoid term. In this review we also focus on the situation of neuroendocrine carcinoma of unk...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - February 28, 2016 Category: Endocrinology Source Type: research

The Syndrome of Inappropriate Antidiuresis (SIAD)
Publication date: Available online 27 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Martin Cuesta, C.J. Thompson Hyponatraemia is the commonest electrolyte disturbance encountered in clinical practice and the syndrome of inappropriate antidiuresis (SIADH) is the most frequent underlying disorder. There is a well-recognized relationship between hyponatraemia and increased morbidity and mortality, though it is unknown whether SIADH confers the same mortality as other causes of hyponatraemia. SIADH is the biochemical manifestation of a wide variety of diseases...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - February 28, 2016 Category: Endocrinology Source Type: research

Diabetes Insipidus in Infants and Children
Publication date: Available online 27 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Elizabeth Dabrowski, Rachel Kadakia, Donald Zimmerman Diabetes insipidus, the inability to concentrate urine resulting in polyuria and polydipsia, can have different manifestations and management considerations in infants and children compared to adults. Central diabetes insipidus, secondary to lack of vasopressin production, is more common in children than is nephrogenic diabetes insipidus, the inability to respond appropriately to vasopressin. The goal of treatment in bot...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - February 28, 2016 Category: Endocrinology Source Type: research

Management of follow-up of neuroendocrine neoplasias
Publication date: Available online 23 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Ulrich-Frank Pape, Sebastian Maasberg, Henning Jann, René Pschowski, Sandrine Krüger, Vikas Prasad, Timm Denecke, Bertram Wiedenmann, Andreas Pascher Neuroendocrine neoplasias (NEN) comprise heterogeneous epithelial neoplasms with a large variety of clinical presentations, treatment options and outcomes. Since potentially all NEN bear malignant potential it is important for long-term clinical management and improvement of outcome to decide on successful an...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - February 23, 2016 Category: Endocrinology Source Type: research

Genetic forms of neurohypophyseal diabetes insipidus
Publication date: Available online 18 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Jonas Rutishauser, Martin Spiess, Peter Kopp Neurohypophyseal diabetes insipidus is characterized by polyuria and polydipsia owing to partial or complete deficiency of the antidiuretic hormone, arginine vasopressin (AVP). Although in most patients non-hereditary causes underlie the disorder, genetic forms have long been recognized and studied both in vivo and in vitro. In most affected families, the disease is transmitted in an autosomal dominant manner, whereas autosomal r...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - February 19, 2016 Category: Endocrinology Source Type: research

Diabetes insipidus: Differential diagnosis and management
Publication date: Available online 18 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Gary L. Robertson Diabetes insipidus (DI) is a syndrome characterized by the excretion of abnormally large volumes of dilute urine. It can be caused by any of 4 fundamentally different defects that must be distinguished for safe and effective management. They are: (1) pituitary DI, due to inadequate production and secretion of antidiuretic hormone, arginine-vasopressin (AVP); (2) gestational DI due to degradation of AVP by an enzyme made in placenta; (3) primary polydipsia, d...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - February 19, 2016 Category: Endocrinology Source Type: research

Copeptin as a biomarker and a diagnostic tool in the evaluation of patients with polyuria-polydipsia and hyponatremia
Publication date: Available online 16 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): M. Christ-Crain, N.G. Morgenthaler, P.D.W. Fenske Copeptin is part of the 164 amino acid precursor protein preprovasopressin together with vasopressin and neurophysin II. During precursor processing, copeptin is released together with vasopressin. Copeptin concentrations respond as rapidly as vasopressin to changes in osmolality, a decrease in blood pressure or stress and there is a close correlation of vasopressin and copeptin concentrations. For these reasons, copeptin is...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - February 17, 2016 Category: Endocrinology Source Type: research

Vasopressin receptor antagonists: characteristics and clinical role
Publication date: Available online 16 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Helbert Rondon-Berrios, Tomas Berl Hyponatremia, the most common electrolyte disorder in hospitalized patients is associated with increased risk of mortality even when mild and apparently asymptomatic. Likewise morbidity manifested as attention deficits, gait disturbances, falls, fractures, and osteoporosis is more prevalent in hyponatremic subjects. Hyponatremia also generates a significant financial burden. Therefore, it is important to explore approaches that effectively ...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - February 16, 2016 Category: Endocrinology Source Type: research

Diabetes Insipidus During Pregnancy
Publication date: Available online 16 February 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Sonia Ananthakrishnan Diabetes insipidus (DI) in pregnancy is a heterogeneous syndrome, most classically presenting with polyuria and polydipsia that can complicate approximately 1 in 30,000 pregnancies. The presentation can involve exacerbation of central or nephrogenic DI during pregnancy, which may have been either overt or subclinical prior to pregnancy. Women without preexisting DI can also be affected by the actions of placental vasopressinase which increases in activit...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - February 16, 2016 Category: Endocrinology Source Type: research

Liver Transplantation for the Treatment of Neuroendocrine Liver Metastases
Publication date: Available online 29 January 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Valery Vilchez, Roberto Gedaly (Source: Best Practice and Research Clinical Endocrinology and Metabolism)
Source: Best Practice and Research Clinical Endocrinology and Metabolism - January 29, 2016 Category: Endocrinology Source Type: research

Gastroenteropancreatic Neuroendocrine Tumours (GEP-NET) – imaging and staging
Publication date: Available online 20 January 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Tobias Baumann, Christof Rottenburger, Guillaume Nicolas, Damian Wild Detection of gastroenteropancreatic neuroendocrine tumours (GEP-NETs) and monitoring of treatment response relies mainly on morphological imaging such as computed tomography (CT) and magnetic resonance imaging (MRI). Molecular imaging techniques also in combination with CT (hybrid imaging) greatly benefit patient management, including better localization of occult tumours and better staging. Somatostatin ...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - January 20, 2016 Category: Endocrinology Source Type: research

Neuroendocrine Tumor Biomarkers: From Monoanalytes to Transcripts and Algorithms
Publication date: Available online 18 January 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Irvin M. Modlin, Lisa Bodei, Mark Kidd The management of neuroendocrine neoplasia remains a perplexing problem because of the lack of knowledge of the biology of the disease, its late presentation, the relative insensitivity of imaging modalities and a paucity of predictably effective treatment options. A critical limitation is posed by the lack of accurate biomarkers to guide management, monitor the efficacy of therapy and provide a prognostic assessment of disease progress...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - January 19, 2016 Category: Endocrinology Source Type: research

Specific biology of neuroendocrine tumors: peptide receptors as molecular targets
Publication date: Available online 15 January 2016 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Meike Körner Neuroendocrine tumors (NET) are characterized by a high over-expression of many different peptide hormone receptors. These receptors represent important molecular targets for imaging and therapy, using either radiolabeled or cold peptide analogs. The clinically best established example is somatostatin receptor targeting. A relatively new application is glucagon-like peptide 1 (GLP-1) receptor-targeted imaging of insulinomas, which is highly sensitive. A poten...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - January 16, 2016 Category: Endocrinology Source Type: research

Diagnosis and treatment of hyponatraemia
Publication date: Available online 30 December 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): S.G. Ball, Zohaib Iqbal Hyponatraemia is the most common electrolyte abnormality encountered by physicians in the hospital setting. It is associated with increased mortality and length of hospital stay. However, the basis of the relationship of hyponatraemia with clinical outcome is not clear. Doubt remains as to whether the relationship is causal. It may reflect the association of two independent variables both of which are linked with disease severity. Serum sodium concent...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - January 13, 2016 Category: Endocrinology Source Type: research

From substitution of insulin to replacement of insulin producing cells: New therapeutic opportunities from research on pancreas development and stem cell differentiation
Publication date: Available online 10 November 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Henryk Zulewski (Source: Best Practice and Research Clinical Endocrinology and Metabolism)
Source: Best Practice and Research Clinical Endocrinology and Metabolism - November 10, 2015 Category: Endocrinology Source Type: research

Inherited defects of thyroxine-binding proteins
Publication date: October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism, Volume 29, Issue 5 Author(s): Theodora Pappa, Alfonso Massimiliano Ferrara, Samuel Refetoff Thyroid hormones (TH) are bound to three major serum transport proteins, thyroxine-binding globulin (TBG), transthyretin (TTR) and human serum albumin (HSA). TBG has the strongest affinity for TH, whereas HSA is the most abundant protein in plasma. Individuals harboring genetic variations in TH transport proteins present with altered thyroid function tests, but are clinically euthyroid and do not require treatment...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - November 6, 2015 Category: Endocrinology Source Type: research

Human pluripotent stem cell based islet models for diabetes research
Publication date: Available online 30 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Diego Balboa, Timo Otonkoski Although similar, mouse and human pancreatic development and beta cell physiology have significant differences. For this reason, mouse models present shortcomings that can obscure the understanding of human diabetes pathology. Progress in the field of human pluripotent stem cell (hPSC) differentiation now makes it possible to derive unlimited numbers of human beta cells in vitro. This constitutes an invaluable approach to gain insight into human b...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - November 1, 2015 Category: Endocrinology Source Type: research

Differentiation of Human Pluripotent Stem Cells into β-cells: Potential and Challenges
Publication date: Available online 30 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Nina Quiskamp, Jennifer E. Bruin, Timothy J. Kieffer Human embryonic stem cells (hESCs) and induced pluripotent stem cells (iPSCs) hold great potential as the basis for cell-based therapies of degenerative diseases, including diabetes. Current insulin-based therapies for diabetes do not prevent hyperglycemia or the associated long-term organ damage. While transplantation pancreatic islets can achieve insulin independence and improved glycemic control, it is limited by donor ...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - November 1, 2015 Category: Endocrinology Source Type: research

A short history of neuroendocrine tumours and their peptide hormones
Publication date: Available online 23 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Wouter W. de Herder, Jens F. Rehfeld, Mark Kidd, Irvin M. Modlin The discovery of neuroendocrine tumours of the gastrointestinal tract and pancreas started in 1870, when Rudolf Heidenhain discovered the neuroendocrine cells, which can lead to the development of these tumours. Siegfried Oberndorfer was the first to introduce the term carcinoid in 1907. The pancreatic islet cells were first described in 1869 by Paul Langerhans. In 1924, Seale Harris was the first to describe ...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 23, 2015 Category: Endocrinology Source Type: research

Implication of epigenetics in pancreas development and disease
Publication date: Available online 23 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Evans Quilichini, Cécile Haumaitre Pancreas development is controlled by a complex interaction of signaling pathways and transcription factor networks that determine pancreatic specification and differentiation of exocrine and endocrine cells. Epigenetics adds a new layer of gene regulation. DNA methylation, histone modifications and non-coding RNAs recently appeared as important epigenetic factors regulating pancreas development. In this review, we report recent findi...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 23, 2015 Category: Endocrinology Source Type: research

Neuroendocrine tumours – Medical therapy: Biological
Publication date: Available online 9 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Anja Rinke, Sebastian Krug Somatostatin analogues (SSA) are well established antisecretory drugs that have been used as first line treatment for symptomatic control in hormonally active neuroendocrine tumours (NET) for three decades. Both available depot formulations of SSA, long-acting repeatable (LAR) octreotide and lanreotide autogel, seem similarly effective and well tolerated, although comparative trials in NET have not been performed. The importance of SSA as antiprolife...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 22, 2015 Category: Endocrinology Source Type: research

Neuroendocrine Tumours- Medical Therapy: Biological
Publication date: Available online 9 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Anja Rinke, Sebastian Krug Somatostatin analogues (SSA) are well established antisecretory drugs that have been used as first line treatment for symptomatic control in hormonally active neuroendocrine tumors (NET) for three decades. Both available depot formulations of SSA, long-acting repeatable (LAR) octreotide and lanreotide autogel, seem similarly effective and well tolerated, although comparative trials in NET have not been performed. The importance of SSA as antiprolifer...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 10, 2015 Category: Endocrinology Source Type: research

Carcinoid Heart Disease: Diagnosis and Management
Publication date: Available online 9 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Sushil A. Luis, Patricia A. Pellikka Hedinger syndrome refers to carcinoid valvular heart disease. The disease is believed to be triggered by vasoactive substances that result in valvular fibrosis. It classically occurs in patients with metastatic carcinoid and preferentially involves the right sided cardiac valves. Affected valves become thickened and retracted, exhibiting regurgitation and sometimes, stenosis. Echocardiography is recommended in patients with carcinoid syndro...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 10, 2015 Category: Endocrinology Source Type: research

Reprogramming of liver cells into insulin-producing cells
Publication date: Available online 8 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Irit Meivar-Levy, Sarah Ferber Tissue replacement is a promising direction for the treatment of diabetes, which will become widely available only when islets or insulin-producing cells that will not be rejected by the diabetic recipients are available in unlimited amounts. The present review addresses the research in the field of generating functional insulin-producing cells by transdifferentiation of adult liver cells both in vitro and in vivo. It presents recent knowledge of...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 9, 2015 Category: Endocrinology Source Type: research

Other miscellaneous hormone binding proteins: attempt at an epilogue
Publication date: Available online 8 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Juergen Kratzsch (Source: Best Practice and Research Clinical Endocrinology and Metabolism)
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 9, 2015 Category: Endocrinology Source Type: research

Peptide Receptor Radionuclide Therapy of Neuroendocrine Tumours
Publication date: Available online 8 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Tessa Brabander, Jaap J.M. Teunissen, Casper H.J. Van Eijck, Gaston J.H. Franssen, Richard A. Feelders, Wouter W. de Herder, Dik J. Kwekkeboom In the past decades the number of neuroendocrine tumours that are detected is increasing. A relative new and promising therapy for patients with metastasised or inoperable disease is peptide receptor radionuclide therapy (PRRT). This therapy involves an infusion of somatostatin analogues linked to radionuclides like Yttrium-90 or L...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 9, 2015 Category: Endocrinology Source Type: research

Surgical Management of Neuroendocrine Tumors
Publication date: Available online 8 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Domenico Tamburrino, Gabriele Spoletini, Stefano Partelli, Francesca Muffatti, Olga Adamenko, Stefano Crippa, Massimo Falconi During the last decades an increase in the incidence of neuroendocrine tumors (NETs) was observed. Gastroenteropancreatic NETs represent the majority of NETs. Compared with their epithelial counterpart they usually have a more indolent behavior and surgical resection improves survival. Tumor diameter is one of the main parameter in the decision mak...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 9, 2015 Category: Endocrinology Source Type: research

Evolving Function and Potential of Pancreatic Alpha Cells
Publication date: Available online 8 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Violeta Stanojevic, Joel F. Habener The alpha cells that co-occupy the islets in association with beta cells have been long recognized as the source of glucagon, a hyperglycemia-producing and diabetogenic hormone. Although the mechanisms that control the functions of alpha cells, glucagon secretion, and the role of glucagon in diabetes have remained somewhat enigmatic over the fifty years since their discovery, seminal findings during the past few years have moved alpha cells ...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 9, 2015 Category: Endocrinology Source Type: research

Somatic and Germline Mutations in Nets: Implications for their Diagnosis and Management
Publication date: Available online 9 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Marianna Minnetti, Ashley Grossman It is now understood that specific somatic and germline mutations may lead to the development of the neuroendocrine tumours (NETs). NETs usually occur as sporadic isolated tumours, although they also may present as part of complex familial endocrine cancer syndromes, such as multiple endocrine neoplasia type 1 and type 2, Von Hippel-Lindau and neurofibromatosis syndromes, tuberous sclerosis, Carney triad and dyad, Reed syndrome and polycythae...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 9, 2015 Category: Endocrinology Source Type: research

Soluble receptors and hormone binding proteins: analytical approach and clinical relevance
Publication date: Available online 9 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Juergen Kratzsch, Wieland Kiess (Source: Best Practice and Research Clinical Endocrinology and Metabolism)
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 9, 2015 Category: Endocrinology Source Type: research

Reprogramming of human exocrine pancreas cells to beta cells
Publication date: Available online 9 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Willem Staels, Yves Heremans, Harry Heimberg (Source: Best Practice and Research Clinical Endocrinology and Metabolism)
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 9, 2015 Category: Endocrinology Source Type: research

Proper activation of MafA is required for optimal differentiation and maturation of pancreatic β-cells
Publication date: Available online 9 October 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Ilham El Khattabi, Arun Sharma A key therapeutic approach for the treatment of Type 1 diabetes (T1D) is transplantation of functional islet β-cells. Despite recent advances in generating stem cell-derived glucose-responsive insulin+ cells, their further maturation to fully functional adult β-cells still remains a daunting task. Conquering this hurdle will require a better understanding of the mechanisms driving maturation of embryonic insulin+ cells into adult β...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 9, 2015 Category: Endocrinology Source Type: research

Inherited defects of thyroxin-binding proteins
Publication date: Available online 30 September 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Theodora Pappa, Alfonso Massimiliano Ferrara, Samuel Refetoff Thyroid hormones (TH) are bound to three major serum transport proteins, thyroxin-binding globulin (TBG), transthyretin (TTR) and human serum albumin (HSA). TBG has the strongest affinity for TH, whereas HSA is the most abundant protein in plasma. Individuals harboring genetic variations in TH transport proteins present with altered thyroid function tests, but are clinically euthyroid and do not require treatmen...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 1, 2015 Category: Endocrinology Source Type: research

Beyond soluble transferrin receptor: Old challenges and new horizons
Publication date: Available online 30 September 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Kristian Harms, Thorsten Kaiser Disturbances of iron metabolism are a frequent challenge in outpatient and inpatient care. Although several established biomarkers are commonly used by clinicians for differential diagnosis, the discrimination between latent or classic iron deficiency, anaemia of chronic disease or a combination of functional iron deficiency (iron-restricted erythropoiesis) with anaemia of chronic disease in patients affected by inflammatory disease can be de...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - October 1, 2015 Category: Endocrinology Source Type: research

Corticosteroid-binding globulin: modulating mechanisms of bioavailability of cortisol and its clinical implications
Publication date: Available online 11 September 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Yoon Ju Bae, Juergen Kratzsch Corticosteroid-binding globulin (CBG) is the principal transport protein of glucocorticoids. Approximately 80-90% of serum cortisol binds to CBG with high affinity and only about 5% of cortisol remain unbound and is considered biologically active. CBG seems to modulate and influence the bioavailability of cortisol to local tissues. In this review, we will discuss physicochemical properties of CBG and structure of CBG in the mechanisms of bindin...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - September 11, 2015 Category: Endocrinology Source Type: research

The soluble leptin receptor
Publication date: Available online 6 September 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Michael Schaab, Juergen Kratzsch The adipokine leptin realizes signal transduction via four different leptin receptor (OB-R) isoforms. The amount of functionally active OB-R, however, is affected by constitutive shedding of the extracellular domain. The product of the cleavage process, the so-called soluble leptin receptor (sOB-R), is the main binding protein for leptin in human blood and modulates its bioavailability. Concentrations of sOB-R are differentially regulated in ...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - September 7, 2015 Category: Endocrinology Source Type: research

Growth hormone binding protein – Physiological and analytical aspects
Publication date: Available online 27 June 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Katharina Schilbach, Martin Bidlingmaier A significant proportion of total circulating growth hormone (GH) is bound to a high affinity growth hormone binding protein (GHBP). Several low affinity binding proteins have also been described. Significant differences between species exist with respect to origin and regulation of GHBP, but generally it resembles the extracellular domain of the GH receptor. Concentrations are associated with GH status, body composition and other factors...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - August 21, 2015 Category: Endocrinology Source Type: research

Insulin-like growth factor binding-protein-3 (IGFBP–3)
Publication date: Available online 27 June 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Michael B. Ranke The IGF system comprises two IGFs (IGF-1, IGF-2), two IGF-receptors (IGF-R1, IGF-R2), and six IGF binding proteins (IGFBPs) with a high affinity for IGFs. The IGFBPs, of which IGFBP-3 is the most abundant in postnatal blood, link with IGFs and prevent them from being degraded; they also facilitate IGF transport through body compartments. The interaction between IGFs and their specific receptors is partly regulated by structural modifications inherent to the IGFBP...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - August 21, 2015 Category: Endocrinology Source Type: research

Insulin-like growth factor binding proteins 4-6
Publication date: Available online 27 June 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Leon A. Bach Insulin-like growth factor binding proteins (IGFBPs) 4-6 have important roles as modulators of IGF actions. IGFBP-4 and IGFBP-6 predominantly inhibit IGF actions, whereas IGFBP-5 may enhance these actions under some circumstances. IGFBP-6 is unique among the IGFBPs for its marked IGF-II binding preference. IGFBPs 4-6 are found in the circulation as binary complexes with IGFs that can enter tissues. Additionally, about half of the circulating IGFBP-5 is found in terna...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - August 21, 2015 Category: Endocrinology Source Type: research

Vitamin D signaling in calcium and bone homeostasis: A delicate balance
Publication date: Available online 30 June 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Geert Carmeliet, Veronique Dermauw, Roger Bouillon Loss-of-function mutations in genes involved in the vitamin D/vitamin D receptor system have clearly evidenced its critical role for mineral and skeletal homeostasis. Adequate levels of 1,25-dihydroxyvitamin D [1,25(OH)2D], the active form of vitamin D are therefore required and depend on sufficient sunlight exposure or dietary intake. Intestinal calcium absorption is a primary target of 1,25(OH)2D action and this pathway indir...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - August 21, 2015 Category: Endocrinology Source Type: research

The biomarker sex hormone-binding globulin – From established applications to emerging trends in clinical medicine
Publication date: Available online 30 June 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Markus A. Thaler, Vanadin Seifert-Klauss, Peter B. Luppa Sex hormone-binding globulin (SHBG) is a serum glycoprotein exhibiting the unique feature of binding sex steroids with high affinity and specificity. Its serum levels are regulated not only by androgens and estrogens but also by thyroid hormones and other metabolic factors. Several disease conditions are accompanied by altered SHBG levels such as hyper- and hypoandrogenism, thyroid disorders, pituitary diseases, liver dis...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - August 21, 2015 Category: Endocrinology Source Type: research

Behind the scenes of vitamin D binding protein: More than vitamin D binding
Publication date: Available online 2 July 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Joris R. Delanghe, Reinhart Speeckaert, Marijn M. Speeckaert Although being discovered in 1959, the number of published papers in recent years reveals that vitamin D binding protein (DBP), a member of the albuminoid superfamily, is a hot research topic. Besides the three major phenotypes (DBP1F, DBP1S and DBP2), more than 120 unique variants have been described of this polymorphic protein. The presence of DBP has been demonstrated in different body fluids (serum, urine, breast m...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - August 21, 2015 Category: Endocrinology Source Type: research

Physiology and pathophysiology of IGFBP-1 and IGFBP-2 – Consensus and dissent on metabolic control and malignant potential
Publication date: Available online 8 July 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Andreas Hoeflich, Vincenzo C. Russo IGFBP-1 and IGFBP-2 are suppressed by growth hormone and therefore represent less prominent members of the IGFBP family when compared to IGFBP-3 that carries most of the IGFs during circulation under normal conditions in humans in vivo. As soon as the GH signal is decreased expression of IGF-I and IGFBP-3 is reduced. Under conditions of lowered suppression by GH the time seems come for IGFBP-1 and IGFBP-2. Both IGFBPs are potent effectors ...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - August 21, 2015 Category: Endocrinology Source Type: research

The soluble interleukin-6 receptor and related proteins
Publication date: Available online 8 July 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Stefan Rose-John Interleukin-6 is a cytokine involved in the regulation of the immune system and the central nervous system. Interleukin-6 binds to an interleukin-6 receptor, and then associates with a dimer of the ubiquitously expressed gp130 receptor subunit, which initiates intracellular signaling. The interleukin-6 receptor is found in a soluble form, which is generated by proteolytic cleavage and also to a minor extent by translation from an alternatively spliced mRNA. The co...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - August 21, 2015 Category: Endocrinology Source Type: research

Noncoding RNAs and the control of signalling via nuclear receptor regulation in health and disease
Publication date: Available online 14 July 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Paul Cathcart, Walter Lucchesi, Silvia Ottaviani, Alex De Giorgio, Jonathan Krell, Justin Stebbing, Leandro Castellano Nuclear receptors belong to a superfamily of proteins that play central roles in human biology, orchestrating a large variety of biological functions in both health and disease. Understanding the interactions and regulatory pathways of NRs will allow development of potential therapeutic interventions for a multitude of disease processes. Non-coding RNAs hav...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - August 21, 2015 Category: Endocrinology Source Type: research

Classical nuclear hormone receptor activity as a mediator of complex biological responses: A look at health and disease
Publication date: Available online 16 July 2015 Source:Best Practice & Research Clinical Endocrinology & Metabolism Author(s): Paul Michael Yen Nuclear hormone receptors are a large family of receptors that bind a wide range of lipolic hormones and intracellular ligands. They act as ligand-inducible transcription factors to regulate the expression of target genes and play important roles in normal development, reproduction, and metabolism. NRs bind to hormones steroids, thyroid hormone, and vitamin D as well as metabolites of fatty acids, cholesterol, and bild acids. Orphan receptors are another group of...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - August 21, 2015 Category: Endocrinology Source Type: research