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33 Cards in this Set
- Front
- Back
CAH
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Congenital adrenal hyperplasia
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CAH inheritance
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Autosomal recessive
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Cause of CAH
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21-hydroxylase deficiency - can't make CORTISOL.
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Results of 21-Hydroxylase deficiency:
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1. Precursors accumulate - shunt to androgen pathway
2. Can't make Aldosterone - Sodium wasting 3. Virilizing |
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Tx for CAH:
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-Replacement therapy
-Glucocorticoids -Aldosterone |
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Why is Cortisol deficiency termed "hyperplasia"?
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-Low cortisol leads to
-High ACTH -Enlarges adrenal gland -Stim pregnenalone synthesis |
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What is Addison's disease?
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Primary adrenal insufficiency
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What causes Addison's?
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Destruction of the adrenal cortex
-by lymphocytes -Tuberculosis |
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Symptoms of Addisons:
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-Fatigue
-Weakness -Weight loss -Salt craving |
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Tx for Addisons:
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Replacement therapy -
Glucocorticoids + Aldosterone |
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What is Cushing's disease?
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Excessive secretion of ACTH by anterior pituitary tumors -> stimulates excessive cortisol production by adrenals.
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How much of Cushing's syndrome is caused by ant pit tumor?
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70%
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Sx of Cushing's:
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-Sudden weight gain
-Muscle wasting -Weak/hypertensive/hyperglycemic |
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Tx for Cushings:
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-Microadenomectomy
-Pituitary irradiation -Adrenalectomy |
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What is the primary mechanism of steroid hormone action?
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Alteration of gene expression
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Where are steroid hormone receptors found?
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-Cytoplasm
-Nucleus |
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What are steroid hormone receptors?
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Ligand-inducible transcription factors
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What do steroid receptors do?
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Bind Hormone response elements (HRE)
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What must happen in order for a steroid hormone receptor to bind its HRE?
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Conformational change induced by ligand (steroid) binding.
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So how do Steroid+Steroid receptor dimers work?
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By acting as transcription factors.
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What is Tamoxifen?
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One of the most widely prescribed compounds for Br. cancer treatment/prevention
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How does Tamoxifen work?
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By selectively binding estrogen receptors and modulating them.
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How does Tamoxifen's binding to estrogen receptor stop cancer?
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By inhibiting other coactivators from binding the regulator complex - gene transcription inhibited.
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What is Tamoxifen an example of?
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A SERM
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what is a serm?
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Selective Estrogen Receptor Modulator
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What is a SERM used to treat osteoporosis?
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Reloxifen
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How do big bulky steroid receptors get into the nucleus?
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Zinc finger proteins act as Nuclear Localizing Sequences (NLS)
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What part of DNA do steroid hormones interact with?
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Major groove
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Major biological effects of Glucocorticoids:
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Protein catabolism
Gluconeogenesis |
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Major biological effects of Mineralcorticoids:
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Na/K regulation
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Major biological effects of Androgens:
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Spermatogenesis
Increased muscle mass |
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Major biological effects of Estrogens:
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Cyclic rythms
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Major biological effects of Progestins:
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Pregnancy
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