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33 Cards in this Set

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