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Related Concept Videos

Anatomy of the Adrenal Glands01:17

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The adrenal or supra-renal glands, situated above the kidneys and aligned with the twelfth rib, are paired pyramid-shaped structures crucial for the body's stress response. During stress, these glands secrete hormones vital for adaptive physiological reactions.
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Adrenal gland disorders manifest when the production of adrenal hormones deviates from the norm, resulting in either excessive or insufficient concentrations.
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Hormones of the Adrenal Glands01:31

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In analyzing the behavior of diodes in circuits, the relationship between the current through a diode and the voltage across it is of particular interest, especially when considering the effect of a direct current (DC) bias voltage. When applied, this DC bias influences the diode's operating point, known as the Q point, around which the current-voltage (I-V) characteristic of the diode exhibits exponential behavior. Introducing a small, time-varying signal on top of this bias aids in examining...
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The response to stress—be it physical or psychological, acute or chronic—involves activation of the Hypothalamic-Pituitary-Adrenal (HPA) axis. The HPA axis is part of the neuroendocrine system because it involves both neuronal and hormonal communication. Its function is to regulate homeostatic systems—metabolic, cardiovascular, and immune—providing the necessary means to respond to a stressor.
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The sympathetic pathways of the collateral ganglia and adrenal medulla serve unique but interconnected roles in the sympathetic response.
Collateral Ganglia
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Isolation, Fixation, and Immunofluorescence Imaging of Mouse Adrenal Glands
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ACTH signalling and adrenal development: lessons from mouse models.

Tatiana V Novoselova1, Peter J King1, Leonardo Guasti1

  • 1Centre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London, UK.

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|June 13, 2019
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Summary
This summary is machine-generated.

Familial glucocorticoid deficiency (FGD) is a rare disease. Mouse models reveal that the melanocortin-2-receptor accessory protein (MRAP) is crucial for adrenal gland development and function.

Keywords:
ACTHMC2RMRAPadrenalstem cells

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Area of Science:

  • Endocrinology and Molecular Biology
  • Adrenal Gland Physiology
  • Genetics and Disease Modeling

Background:

  • The melanocortin-2-receptor (MC2R), or ACTH receptor, is vital for the hypothalamic-pituitary-adrenal axis.
  • Familial glucocorticoid deficiency (FGD) results from isolated cortisol deficiency, with MC2R mutations causing ~25% of cases.
  • MC2R accessory protein (MRAP) mutations account for ~20% of FGD, highlighting its role in MC2R function.

Purpose of the Study:

  • To investigate the role of MRAP in adrenal physiology using mouse models.
  • To compare the utility of Mc2r-knockout and Mrap-knockout mouse models in studying FGD.
  • To elucidate the impact of MRAP deficiency on adrenal gland development and signaling pathways.

Main Methods:

  • Generation and analysis of Mc2r-knockout and Mrap-knockout mouse models.
  • Phenotypic characterization of adrenal glands in knockout mice.
  • Assessment of WNT4/beta-catenin and sonic hedgehog (SHH) pathway signaling.

Main Results:

  • Mc2r-knockout mice exhibited deficiencies in glucocorticoid, mineralocorticoid, and catecholamines.
  • Mrap-knockout mice accurately mimicked the human FGD phenotype with isolated glucocorticoid deficiency.
  • Mrap-knockout mice displayed adrenal dysmorphology, including thickened capsules, disrupted zonation, and altered WNT4/beta-catenin and SHH signaling.

Conclusions:

  • MRAP is essential for MC2R expression and function, critical for adrenal gland development.
  • Mrap-knockout mice provide a superior model for studying isolated glucocorticoid deficiency (FGD).
  • ACTH and MRAP are crucial for regulating adrenal progenitor cells, cortex maintenance, and zonation.