Hyperinsulinaemic Hypoglycaemia - The Molecular Mechanisms
Under normal physiological conditions pancreatic β-cells secrete insulin to maintain fasting blood glucose levels in the range 3.5-5.5mmol/L. In hyperinsulinaemic hypoglycaemia (HH) this precise regulation of insulin secretion is perturbed so that insulin continues to be secreted in the presence of...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2016-03-01
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| Series: | Frontiers in Endocrinology |
| Subjects: | |
| Online Access: | http://journal.frontiersin.org/Journal/10.3389/fendo.2016.00029/full |
| _version_ | 1811298484355072000 |
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| author | Azizun eNessa Sofia A Rahman Khalid eHussain Khalid eHussain |
| author_facet | Azizun eNessa Sofia A Rahman Khalid eHussain Khalid eHussain |
| author_sort | Azizun eNessa |
| collection | DOAJ |
| description | Under normal physiological conditions pancreatic β-cells secrete insulin to maintain fasting blood glucose levels in the range 3.5-5.5mmol/L. In hyperinsulinaemic hypoglycaemia (HH) this precise regulation of insulin secretion is perturbed so that insulin continues to be secreted in the presence of hypoglycaemia. HH may be due to genetic causes (congenital) or secondary to certain risk factors. The molecular mechanisms leading to HH involve defects in the key genes regulating insulin secretion from the β-cells. At this moment in time genetic abnormalities in 9 genes (ABCC8, KCNJ11, GCK, SCHAD, GLUD1, SLC16A1, HNF1A, HNF4A and UCP2) have been described that lead to the congenital forms of HH. Perinatal stress, intrauterine growth retardation, maternal diabetes mellitus and a large number of developmental syndromes are also associated with HH in the neonatal period. In older children and adult’s insulinoma, Noninsulinoma pancreatogenous hypoglycaemia syndrome and post bariatric surgery are recognised causes of HH. This review article will focus mainly on describing the molecular mechanisms that lead to unregulated insulin secretion. |
| first_indexed | 2024-04-13T06:20:05Z |
| format | Article |
| id | doaj.art-75baf8ea35284b189368975d7536ef19 |
| institution | Directory Open Access Journal |
| issn | 1664-2392 |
| language | English |
| last_indexed | 2024-04-13T06:20:05Z |
| publishDate | 2016-03-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Endocrinology |
| spelling | doaj.art-75baf8ea35284b189368975d7536ef192022-12-22T02:58:41ZengFrontiers Media S.A.Frontiers in Endocrinology1664-23922016-03-01710.3389/fendo.2016.00029182001Hyperinsulinaemic Hypoglycaemia - The Molecular MechanismsAzizun eNessa0Sofia A Rahman1Khalid eHussain2Khalid eHussain3UCL Institute of Child HealthUCL Institute of Child HealthUCL Institute of Child HealthGreat Ormond Street Hospital for ChildrenUnder normal physiological conditions pancreatic β-cells secrete insulin to maintain fasting blood glucose levels in the range 3.5-5.5mmol/L. In hyperinsulinaemic hypoglycaemia (HH) this precise regulation of insulin secretion is perturbed so that insulin continues to be secreted in the presence of hypoglycaemia. HH may be due to genetic causes (congenital) or secondary to certain risk factors. The molecular mechanisms leading to HH involve defects in the key genes regulating insulin secretion from the β-cells. At this moment in time genetic abnormalities in 9 genes (ABCC8, KCNJ11, GCK, SCHAD, GLUD1, SLC16A1, HNF1A, HNF4A and UCP2) have been described that lead to the congenital forms of HH. Perinatal stress, intrauterine growth retardation, maternal diabetes mellitus and a large number of developmental syndromes are also associated with HH in the neonatal period. In older children and adult’s insulinoma, Noninsulinoma pancreatogenous hypoglycaemia syndrome and post bariatric surgery are recognised causes of HH. This review article will focus mainly on describing the molecular mechanisms that lead to unregulated insulin secretion.http://journal.frontiersin.org/Journal/10.3389/fendo.2016.00029/fullGlucoseInsulinKATP Channelscongenital hyperinsulinismHyperinsulinaemic hypoglycaemia |
| spellingShingle | Azizun eNessa Sofia A Rahman Khalid eHussain Khalid eHussain Hyperinsulinaemic Hypoglycaemia - The Molecular Mechanisms Frontiers in Endocrinology Glucose Insulin KATP Channels congenital hyperinsulinism Hyperinsulinaemic hypoglycaemia |
| title | Hyperinsulinaemic Hypoglycaemia - The Molecular Mechanisms |
| title_full | Hyperinsulinaemic Hypoglycaemia - The Molecular Mechanisms |
| title_fullStr | Hyperinsulinaemic Hypoglycaemia - The Molecular Mechanisms |
| title_full_unstemmed | Hyperinsulinaemic Hypoglycaemia - The Molecular Mechanisms |
| title_short | Hyperinsulinaemic Hypoglycaemia - The Molecular Mechanisms |
| title_sort | hyperinsulinaemic hypoglycaemia the molecular mechanisms |
| topic | Glucose Insulin KATP Channels congenital hyperinsulinism Hyperinsulinaemic hypoglycaemia |
| url | http://journal.frontiersin.org/Journal/10.3389/fendo.2016.00029/full |
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