hrp0089fc14.4 | Multisystem Endocrine Disorders | ESPE2018

Glucocorticoid Deficiency Causes Differentially Dysregulated Oxidative Stress Depending on the Steroidogenic Defects

Li Nan , Weger Meltem , Griffin Aliesha , Eachus Helen , Cunliffe Vincent T , Krone Nils

Glucocorticoids regulate a wide range of biological processes including metabolism. Patients with adrenal insufficiency show impaired glucocorticoid biosynthesis either caused by adrenal defects (primary adrenal insufficiency) or by defects in the pituitary gland or hypothalamus (secondary or tertiary adrenal insufficiency). The systemic consequences of differentially disrupted steroid hormone biosynthesis remain unclear. Increasing evidence suggested steroid hormone precursor...

hrp0086rfc1.2 | Adrenals | ESPE2016

Glucocorticoid Deficiency Due to Disruption of Mitochondrial Steroidogenesis Leads to Dysregulation of Antioxidant Pathways and Nucleotide Biosynthesis

Weger Meltem , Gorling Benjamin , Poschet Gernot , Griffin Aliesha , Hell Rudiger , Luy Burkhard , Muller Ferenc , Krone Nils

Glucocorticoids are important regulators of systemic homeostasis. However, the role of these steroid hormones has been mainly studied by using synthetic glucocorticoids or in states of glucocorticoid excess. Thus, the pathophysiologic consequences of cortisol deficiency on metabolic and biosynthesis pathways remain largely elusive. Zebrafish is a well-established vertebrate model for studying whole organism biology. Similar to humans, zebrafish are day active and the key gluco...

hrp0086rfc1.5 | Adrenals | ESPE2016

A Novel Animal Model to Study 21-Hydroxylase Deficiency in vivo

Zaucker Andreas , Griffin Aliesha , Storbeck Karl-Heinz , Guran Tulay , Thakur Nazia , Weger Meltem , Taylor Angela , Mueller Ferenc , Krone Nils

Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD) is caused by mutations in the CYP21A2 gene. Steroid 21-hydroxylase deficiency results in impaired synthesis of mineralcorticoids and glucocorticoids (GC), plus androgen excess. Hormonal imbalances in 21OHD are postulated to result in systemic transcriptomic and metabolomic alterations. Such perturbations are likely to be underlying co-morbidities, which are increasingly observed in individua...