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35 Cards in this Set

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What is correct about fats?
They are mainly produced in the liver.
Which of the following is the best source of unsaturated fatty acids?
vegetable oils
Insulin activates hormone-sensitive lipase and triggers TAGs degradation.
False: insulin is low when the FA is released and epinepherine activates the enzyme. high insulin we don't need FA.
Linolenic Deficiency
decreased vision and altered learning behaviors
CPT-I deficiency (carnitine shuttle)
that affects the liver’s ability to synthesize glucose during a fast
Can lead to severe hypoglycemia, coma, and death
CPT-II deficiency
that occurs primarily in cardiac and skeletal muscle
Causes cardiomyopathy, muscle weakness + myoglobinemia following prolonged exercise
Liver disease, malnutrition or strictly vegetarian diet
Increased requirement in carnitine during pregnancy, severe infection, or trauma
Hemodialysis, which removes carnitine from the blood
Treatment: avoid prolonged fast, low LCFA and high carbohydrate diet, supplemented with MCFA and, if necessary, carnitine supplement
MCAD Deficiency
Medium-chain fatty acyl-CoA dehydrogenase (MCAD) deficiency is the most common inborn error of FA metabolism
Decrease in FA oxidation → severe hypoglycemia
Infants are particularly affected by MCAD deficiency
Breast milk contains primarily MCFAs
Treatment includes high-carbohydrate diet
Excessive ketone body formation
High ketone bodies level in blood (ketonemia) and in urine (ketonuria)
Most often in Type 1 (insulin-dependent) diabetes

High FA degradation -> high acetyl CoA and low NAD+ -> slows TCA -> forces acetyl CoA to ketone bodies
Severe ketosis – urinary excretion can reach 5000mg/24hr, in blood 90 mg/dl (normal 3mg/dl)
Elevated ketone body concentration -> acidemia or ketoacidosis
Ketoacidosis may also happened during fasting
Respiratory distress syndrome (RDS)
Deficient in Dipalmitoyl phosphotidylcholine production. in pre-term infants is associated with insufficient surfactant production
RDS can occur in adults whose surfactant-producing pneumocytes are damaged or destroyed
Niemann-Pick Disease
Autosomal recessive
Deficiency of sphingomyelinase
Severe infantile form (A)
Deposits in the liver and brain (neurodegeneration-Mental retardation)
Death in early childhood
Less severe (B)
Deposits in the liver, spleen, lungs and bone marrow
Death in early adulthood
Incidence in Ashkenazi Jewish population: A – 1:40.000, B-80.000
General population: 1:100.000
Infintile form More severe (A) Niemann pick disease
lipid Deposits in the liver and brain (neurodegeneration)
Death in early childhood
Less severe (B) Niemann pick disease
lipid Deposits in the liver, spleen, lungs and bone marrow. Spleen and liver enlarged-filled with lipids
Death in early adulthood
Lliver is the only site of cholesterol synthesis
false
Which of the following agents is an activator of HMG-CoA reductase?
Insulin-it is the product of high energy
Sphingolipidoses
Diseases of the degradation of glycosphingolipids.
Characteristics:

Caused by deficiency of hydrolases
Leads to accumulation of sphingolipids in lysosomes
Can lead to neurologic deterioration and death
Specific hydrolytic enzyme is deficient in each disorder
Single sphingolipid accumulates in each disease
Disorders are progressive and many fatal in childhood
Most are autosomal recessive
Fabry is X-linked
Incidence is low, except Gaucher and Tay-Sachs diseases
Gaucher Disease
Sphingolipidoses:
Macrophages engulfed with glucocerebroside.
Most common lysosomal storage disease
Results in: Hepatosplenomegaly, Osteoporosis of the long bone, CNS in Juvenile form.
Tay Sachs Disease
Sphingolipidoses:
Accumulation of Gangliosides(GM), lacks hexoaminase
Results in: Neurodegeneration, cherry red macula, Blindness, muscle weakness and seizures
Fabry Disease
Sphingolipidoses: X-linked
Globosides accumulate in vascular lysosomes in brain, heart, kidney, and skin
Results in: Kidney and heart failure, Reddish-purple skin rash, burning pain in lower extremeties
Niemann-Pick Disease
Sphingolipidoses:
Accumulation of Sphingomyelin.
Results In: Hepatosplenomegaly, Neurodegeneration
Farber Disease
Sphingolipidoses: Accumulation of Ceremides-very rare
Results in: Painful joint deformity, granulomas(tissues)
Krabbe Disease
Sphingolipidoses: Accumulation of galactocerebrosides.
Results in: Mental and motor deterioration, Blindness, deafness, Near total loss of myelin, Globoid bodies in brain
Metachromatic Leukodystrophy
Sphingolipidoses: Accumulation of Sulfatides,
Results in: Cognitive deterioration, Demylenation, Progressive paralysis, Dementia, nerves are stained yellow
Bile acid sequestrants
not FDA approved and are used to lower cholesterol
Prevent bile acids reabsorption in the gut
The sequestered bile acids are then excreted in the feces. Causes an inability to absorb fat soluble vitamins and drugs.
Gall Stones
Cholesterol solubility is maintained by phospholipids and bile salts
If cholesterol to phospholipid ratio in bile more than 1:1, it results in crystallization of excess cholesterol
Gallstones occur in up to 20% of population in western countries
Cholesterol stones account for about 80 percent of gallstones
Hyperaldosteronism
Primary (Conn’s disease) is caused by aldosterone-secreting tumor. Secondary due to kidney or liver disease is more common
too much aldosterone
Primary adrenal insufficiency or Addison’s disease
Insufficient production of aldosterone and cortisol due to atrophy of adrenal glands (autoimmune disorder, tuberculosis)
Secondary adrenal insufficiency
No enough production of ACTH (abrupt discontinue of taking synthetic glucocorticoids)-steriod therapy
Cushing’s syndrome
caused by excessive amounts of cortisol
Exogenous (iatrogenic) caused by administration of glucocorticoids
Endogenous caused by excessive production of cortisol due to pituitary adenoma and adrenal adenoma/carcinoma
21 alpha hydroxylase deficiency
Steroid hormone synthesis disease. No mineralcorticoids and glucocorticoids present. No aldosterone or cortisol production. Most common form of CAH. Androgen overproduction= external genetalia in females and early virilization in males
17 alpha hydroxylase deficiency
Steroid hormone synthesis disease. No sex hormones(androgens/estrogens) or cortisol produced. Increased minerocorticoid production=Na and fluid retention, hypertension and female like genetalia
Anabolic steroids
Dihydrotestosterone (DHT)

Testosterone (esters)

Methandrostenolone (Dianabol, DBOL)

Oxandrolone (Oxandrin)
Methandrostenolone (Dianabol, DBOL)
Aromatization converts it to estrogen
Heavily masculinising effects
Causes lever damage as all 17α-alkylated steroids.
Androgens aromatized into estrogen=promotes muscle building and glycolysis and female like effects
Oxandrolone (Oxandrin)
Absence of aromatization affect
Does not inhibit normal testosterone production
Causes lever damage as all 17α-alkylated steroids. Androgens not converted to estrogens because of oxygen presence, no female like effects but shrinkage of testicles.
Negative Side Affects of Anabolic Steroids(male)
Baldness
Headaches
Breast development
Enlarged prostate
reduced sperm count
testicle shrinkage
liver damage
strokes and blood clots
Negatuve Side Affects of Anabolic Steroids(female)
Reduced breast size
enlarged clitoris
facial hair and body hair increase
deepened voice menstrual problems