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Biochemistry
Steroid Hormones
27
Other
Graduate
02/27/2009

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Term
Type I (familial LPL deficiency, familial hyperchylomicronemia
Definition
(a) deficiency of LPL; (b) production of abnormal LPL; (c) apoC-II deficiency; Slow chylomicron clearance, reduced LDL and HDL levels; treated by low fat/complex carbohydrate diet; no increased risk of coronary artery disease
Term
Type II (Familial hypercholesterolemia, FH)
Definition
LDL receptor defect, Reduced LDL clearance leads to hypercholesterolemia, resulting in athersclerosis and coronary artery disease
Term
Type III (familial dysbetalipoproteinemia,
Definition
this disease is caused by the apoE2 isoform that interacts poorly with the apoE receptor; Causes xanthomas, hypercholesterolemia and athersclerosis in peripheral and coronary arteries due to elevated levels of chylomicrons and VLDLs
Term
Type IV Familial hypertriacylglycerolemia
Definition
Elevated production of VLDL associated with glucose intolerance and hyperinsulinemia; Frequently associated with type-II non-insulin dependent diabetes mellitus, obesity, alcoholism or administration of progestational hormones
Term
Familial hyperalphalipoproteinemia
Definition
Increased level of HDLs A rare condition that is beneficial for health and longevity
Term
Familial LCAT deficiency
Definition
Absence of LCAT leads to inability of HDLs to take up cholesterol (reverse cholesterol transport) Decreased levels of plasma cholesteryl esters and lysolecithin; abnormal LDLs (Lp-X) and VLDLs; symptoms also found associated with cholestasis
Term
Wolman's disease
Definition
Defect in lysosomal cholesteryl ester hydrolase; affects metabolism of LDLs Reduced LDL clearance leads to hypercholesterolemia, resulting in athersclerosis and coronary artery disease
Term
Abeta lipoproteinemia (acanthocytosis, Bassen-Kornzweig syndrome)
Definition
No chylomicrons, VLDLs or LDLs due to defect in apoB expression Rare defect; intestine and liver accumulate, malabsorption of fat, retinitis pigmentosa, ataxic neuropathic disease, have thorny appearance
Term
Familial alpha-lipoprotein deficiency (Tangier disease, Fish-eye disease, apoA-I and -C-III deficiencies)
Definition
All of these related syndromes have reduced HDL concentrations, no effect on chylomicron or VLDL production Tendency to hypertriacylglycerolemia; some elevation in VLDLs; Fish-eye disease characterized by severe corneal opacity
Term
desmolase
Definition
when cholesterol is degraded to pregnenolone what is the enzyme used that is part of the cholesterol side chain cleavage system
Term
transcortin
Definition
Corticosteroid binding globulin ______carries cortisol
Term
Steroidogenic Acute Regulatory Protein
Definition
Transport of free cholesterol from the cytoplasm into mitochondria.
carried out by the
Term
acetate
Definition
what is the precursor for cholesterol
Term
ACTH
Definition
what stimulates the conversion in the first stage of steroid hormone synthesis
Term
testosterone
Definition
reduction of the 17-keto group of androstenedione produces?
Term
DHT
Definition
Testosterone reduced by 5a-reductase to yield
Term
estrogens
Definition
what are from androgens by the loss of C-19 angular methyl group and the formation of an aromatic A ring.
Term
progesterone
Definition
Maintains pregnancy
Inhibits follicular maturation and ovulation
prevents spontaneous uterine contraction
Term
angiotensin II
Definition
increased conversion of corticosterone to aldosterone in the zona glomerulosa
Term
aldosterone
Definition
Increases renal Na+ reabsorption  restoring extracellular fluid volume and blood volume
Increases renal K+ secretion in the face of hyperkalemia
Term
CRH
Definition
Hypothalamic control is via ____  on pituitary
Term
glucocorticoids
Definition
these are
Important for stress response
Stimulates gluconeogenesis
Increase protein catabolism/decrease synthesis thus providing more amino acids to liver
Decreases glucose use and insulin sensitivity in fat tissue
Increase lipolysis
Term
lipocortin
Definition
Glucocorticoids Induce synthesis of _______ which inhibits phospholipase A2
Inhibits production of IL-2 as well as T-cell production
Inhibits release of histamine and serotonin from mast cells and platelets
Term
21-hydroxylase (CYP21A2) deficiency
Definition
Most common form of CAH accounting for 90% of all cases
Clinical manifestations result from loss of aldosterone and cortisol with precursors being shunted into androgen synthesis
Total loss of enzyme activity results in 'salt-wasting' disease (loss of aldosterone) and virilization and ambigous genitalia of a female infant (increased testosterone production
Term
11β hydroxylase deficiency
Definition
virilization, hypertension, perhaps due to increased production of 11-deoxycortisol which has mineralocorticoid actions
Term
17α hydroxylase deficiency
Definition
hypertension with hypokalemia due to excessive production of mineralocorticoids; failure of pubertal development in genetic females and genetic males presenting at puberty with female external genitalia and intra-abdominal testes
Term
3 β hydroxy steroid dehydrogenase deficiency
Definition
defective production of all steroids.  adrenal failure in early infancy
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