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      Serum sex hormone-binding globulin levels are reduced and inversely associated with intrahepatic lipid content and saturated fatty acid fraction in adult patients with glycogen storage disease type 1a

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          Abstract

          Purpose

          De novo lipogenesis has been inversely associated with serum sex hormone-binding globulin (SHBG) levels. However, the directionality of this association has remained uncertain. We, therefore, studied individuals with glycogen storage disease type 1a (GSD1a), who are characterized by a genetic defect in glucose-6-phosphatase resulting in increased rates of de novo lipogenesis, to assess the downstream effect on serum SHBG levels.

          Methods

          A case–control study comparing serum SHBG levels in patients with GSD1a ( n = 10) and controls matched for age, sex, and BMI ( n = 10). Intrahepatic lipid content and saturated fatty acid fraction were quantified by proton magnetic resonance spectroscopy.

          Results

          Serum SHBG levels were statistically significantly lower in patients with GSD1a compared to the controls ( p = 0.041), while intrahepatic lipid content and intrahepatic saturated fatty acid fraction—a marker of de novo lipogenesis—were significantly higher in patients with GSD1a ( p = 0.001 and p = 0.019, respectively). In addition, there was a statistically significant, inverse association of intrahepatic lipid content and saturated fatty acid fraction with serum SHBG levels in patients and controls combined ( β: − 0.28, 95% CI: − 0.47;− 0.09 and β: − 0.02, 95% CI: − 0.04;− 0.01, respectively).

          Conclusion

          Patients with GSD1a, who are characterized by genetically determined higher rates of de novo lipogenesis, have lower serum SHBG levels than controls.

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          Most cited references36

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          World Medical Association Declaration of Helsinki: ethical principles for medical research involving human subjects.

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            Using human genetics to understand the disease impacts of testosterone in men and women

            Testosterone supplementation is commonly used for its effects on sexual function, bone health and body composition, yet its effects on disease outcomes are unknown. To better understand this, we identified genetic determinants of testosterone levels and related sex hormone traits in 425,097 UK Biobank study participants. Using 2,571 genome-wide significant associations, we demonstrate the genetic determinants of testosterone levels are substantially different between sexes, and that genetically higher testosterone is harmful for metabolic diseases in women but beneficial in men. For example, a genetically determined 1-standard deviation higher testosterone increases the risks of Type 2 diabetes (T2D) (OR=1.37 [1.22–1.53]) and polycystic ovary syndrome (OR=1.51 [1.33–1.72]) in women, but reduces T2D risk in men (OR=0.86 [0.76–0.98]). We also show adverse effects of higher testosterone on breast and endometrial cancers in women, and prostate cancer in men. Our findings provide insights into the disease impacts of testosterone and highlight the importance of sex-specific genetic analyses.
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              The role of hepatokines in metabolism.

              The liver is known to be involved in the natural history of the ongoing epidemics of type 2 diabetes mellitus and cardiovascular disease. In particular, the liver has a role in increased glucose production and dysregulated lipoprotein metabolism, conditions that are often found in patients with nonalcoholic fatty liver disease. Additionally, several proteins that are exclusively or predominantly secreted from the liver are now known to directly affect glucose and lipid metabolism. In analogy to the functional proteins released from adipose tissue and skeletal muscle-adipokines and myokines-these liver-derived proteins are known as hepatokines. The first hepatokine that has been proven to have a major pathogenetic role in metabolic diseases is α2-HS-glycoprotein (fetuin-A). Production of this glycoprotein is increased in steatotic and inflamed liver, but not in expanded and dysregulated adipose tissue. Thus, research into this molecule and other hepatokines is expected to aid in differentiating between the contribution of liver and those of skeletal muscle and adipose tissue, to the pathogenesis of type 2 diabetes mellitus and cardiovascular disease.
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                Author and article information

                Contributors
                mcgj.brouwers@mumc.nl
                Journal
                J Endocrinol Invest
                J Endocrinol Invest
                Journal of Endocrinological Investigation
                Springer International Publishing (Cham )
                0391-4097
                1720-8386
                7 February 2022
                7 February 2022
                2022
                : 45
                : 6
                : 1227-1234
                Affiliations
                [1 ]GRID grid.412966.e, ISNI 0000 0004 0480 1382, Division of Endocrinology and Metabolic Diseases, Department of Internal Medicine, , Maastricht University Medical Centre, ; PO Box 5800, 6202 AZ Maastricht, The Netherlands
                [2 ]GRID grid.5012.6, ISNI 0000 0001 0481 6099, Laboratory for Metabolism and Vascular Medicine, , Maastricht University, ; Maastricht, The Netherlands
                [3 ]GRID grid.5012.6, ISNI 0000 0001 0481 6099, CARIM School for Cardiovascular Diseases, Maastricht University, ; Maastricht, The Netherlands
                [4 ]GRID grid.412966.e, ISNI 0000 0004 0480 1382, Department of Reproductive Medicine, , Maastricht University Medical Centre, ; Maastricht, The Netherlands
                [5 ]GRID grid.5012.6, ISNI 0000 0001 0481 6099, Department of Nutrition and Movement Sciences, , Maastricht University, ; Maastricht, The Netherlands
                [6 ]GRID grid.5012.6, ISNI 0000 0001 0481 6099, Department of Radiology and Nuclear Medicine, , Maastricht University, ; Maastricht, The Netherlands
                [7 ]GRID grid.412966.e, ISNI 0000 0004 0480 1382, Central Diagnostic Laboratory, , Maastricht University Medical Centre, ; Maastricht, The Netherlands
                [8 ]GRID grid.4830.f, ISNI 0000 0004 0407 1981, Section of Metabolic Diseases, , Beatrix Children’s Hospital, University Medical Centre Groningen, University of Groningen, ; Groningen, The Netherlands
                [9 ]GRID grid.412966.e, ISNI 0000 0004 0480 1382, Division of General Internal Medicine, Department of Internal Medicine, , Maastricht University Medical Centre, ; Maastricht, The Netherlands
                Article
                1753
                10.1007/s40618-022-01753-2
                9098618
                35132570
                f67dfbdf-861f-4809-b533-2e147a5b6a0a
                © The Author(s) 2022

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

                History
                : 26 November 2021
                : 22 January 2022
                Funding
                Funded by: European Foundation for the Study of Diabetes
                Funded by: ERC starting grant
                Award ID: 759161 ‘MRS in diabetes’
                Award Recipient :
                Categories
                Original Article
                Custom metadata
                © The Author(s), under exclusive licence to Italian Society of Endocrinology (SIE) 2022

                glycogen storage disease type 1a,de novo lipogenesis,intrahepatic lipid content,sex hormone-binding globulin

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