Lipids, amino acids, and nitrogen

Question 1

ApoB-48

Answer 1

-Chylomicron secretion from enterocyte to lymphatic system

Question 2

ApoB-100

Answer 2

• think mainly VLDL . . mediates secretion
• also on IDL and LDL
• leave liver

Question 3

ApoE

Answer 3

mediates extra remnant uptake

Question 4

ApoA-1

Answer 4

• Activates LCAT

- found on HDL

Question 5

ApoC-II

Answer 5

cofactor for lipoprotein lipase

Question 6

Describe Abetalipoproteinemia

Answer 6

• autosomal recessive
• decrease ApoB-48 and ApoB-100
• mutation of MTP gene
• decrease chylomicron and VLDL synthesis and secretion

Question 7

What is the presentation of Abetalipoproteinemia

Answer 7

• Steatorrhea
• ADEK deficiency
• Intestinal biopsy: enterocytes swollen with TGs
• Peripheral smear: Acantholysis of RBCs
• Ataxia
• NIGHT BLINDNESS

Question 8

What is the precursor molecule for Fatty acid synthesis

Answer 8

-Acetyl CoA

Question 9

location for Fatty acid synthesis

Answer 9

cytoplasm of hepatocytes

Question 10

Rate limiting enzyme for fatty acid synthesis

Answer 10

-Acetyl-CoA carboxylase

Question 11

Location for Fatty acid degradation

Answer 11

Mitochondria

Question 12

Rate limiter for Fatty acid degradation

Answer 12

-Carnitine Acyltransferase I (Carnitine Palmitoyltransferase I)

Question 13

Treatment for Abetalipoproteinemia

Answer 13

Vitamin E

Question 14

Rate limiter for cholesterol synthesis

Answer 14

HMG-CoA Reductase

Question 15

What are the Dyslipidemias?

Answer 15

• Type I hyperchylomicronemia
• Type IIa familial hypercholesterolemia
• Type IV hypertriglyceridemia

Question 16

Describe Type I hyperchylomicronemia

Answer 16

• Autosomal recessive
• Lipoprotein lipase deficiency or defective ApoC-II
• increase in chylomicrons, TGs, cholesterol
• NO INCREASE RISK FOR ATHEROSCLEROSIS

Question 17

Presentation of type I hyperchylomicronemia

Answer 17

• Pancreatitis: from high triglycerides
• Hepatosplenomegaly
• Pruritic Xanthomas

Question 18

Describe type IIa familial hypercholesterolemia

Answer 18

• Autosomal dominant
• Absent or Low LDL receptors
• Presentation: Tendinous Xanthomas
• Corneal Arcus
• ACCELERATED atherosclerosis
• MI

Question 19

Describe Type IV hypertriglyceridemia

Answer 19

• Autosomal dominant
• Overproduction of VLDL
• Pancreatitis

Question 20

What are the essential Amino acids

Answer 20

• “PVT TIM HaLL”
• Phenylalanine
• Valine
• Threonine
• Tryptophan
• Isoleucine
• Methionine
• Histidine
• Leucine
• Lysine

Question 21

What are the basic amino acids and their uses

Answer 21

• Lysine and arginine (extra ammonia group)
• Histidine
• Arginine and Histidine (during periods of growth)
• Arginine and Lysine (nuclear localization signals and high concentrations in histones)

Question 22

What are the acidic Amino acids

Answer 22

-Aspartate and glutamate . . negatively charged

Question 23

What does phenylalanine give rise to

Answer 23

• Tyrosine
• Dopamine
• NE
• Epi

Question 24

What are the Arginine Derivatives

Answer 24

• creatine
• Urea
• Nitric oxide

Question 25

What are the tryptophan derivatives

Answer 25

- Niacin --> NAD+/NADP (B6 cofactor) | - Serotonin --> melatonin

Question 26

Histidine derivative

Answer 26

-histamine (B6 cofactor)

Question 27

Glycine derivative

Answer 27

-----> porphyrin (B6 cofactor) -----> Heme

Question 28

Glutamate derivatives

Answer 28

- GABA (B6 cofactor) | - Glutathione

Question 29

Describe Ornithine Transcarbamoylase (OTC) deficiency

Answer 29

- Most common urea cycle disorder - X-linked recessive - Orotic acid in blood and urine - decreased BUN - Hyper ammonemia--->hepatoencephalopathy: Slurring of speech, somnolence, vomiting, cerebral edema, Blurring of vision

Question 30

Treatment of OTC deficiency

Answer 30

- Low protein diet - Phenylbutyrate - Benzoate - Biotin

Question 31

What is the precursor to S-adenosyl-methionine (SAM)

Answer 31

methionine