Biochem Pages 82-91

Question 1

Patient complaints that he can’t smell well and has a persistently rancid metallic taste in his mouth. On physical exam, there is evidence of hypogonadism, sparse pubic, facial, and axillary hair. What deficiency is suspected?

Answer 1

Zinc

2016; Pg 82

Question 2

Acrodermatitis enteropathica is associated with which vitamin deficiency?

Answer 2

Zinc

2016; Pg 82

Question 3

Malnourished child presents with a swollen abdomen, anemia, and impaired liver function. What disorder is present?

Answer 3

Kwashiorkor
(2016; Pg 82)

Edema is due to decreased oncotic pressure and liver malfunction to decreased apolipoprotein synthesis

Question 4

Total caloric malnutrition resulting in tissue and muscle wasting and loss of subcutaneous fat.

Answer 4

Marasmus

2016; Pg 82

Question 5

Antidote for methanol or ethylene glycol poisoning that Inhibits alcohol dehydrogenase

Answer 5

Fomepizole

2016; Pg 82

Question 6

Drug given to discourage drinking that inhibits acetaldehyde dehydrogenase

Answer 6

Disulfiram

2016; Pg 83

Question 7

Effect of chronic alcoholism on NADH/NAD+ ratio in liver

Answer 7

Increase

2016; Pg 83

Question 8

Chronic intake of _____ is known to induce lactic acidosis, fasting hypoglycemia, and hepatosteatosis.

Answer 8

Ethanol

2016; Pg 83

Question 9

Cellular site for fatty acid beta oxidation, acetyl-CoA production, TCA cycle, oxidative phosphorylation, and ketogenesis.

Answer 9

Mitochondria

2016; Pg 83

Question 10

Cellular site for glycolysis, HMP shunt, and the synthesis of steroids, proteins, fatty acids, cholesterol, and nucleotides.

Answer 10

Cytoplasm

2016; Pg 83

Question 11

Where do heme synthesis, the urea cycle, and gluconeogenesis occur?

Answer 11

Both the mitochondria and cytoplasm
(2016; Pg 83)

HUGs take two

Question 12

Relocates a functional group within a molecule

Answer 12

Mutase

2016; Pg 84

Question 13

Co-factor in the transfer of CO2 groups by carboxylases

Answer 13

Biotin

2016; Pg 84

Question 14

Rate-determining enzyme of glycolysis

Answer 14

Phosphofructokinase-1 (PFK-1)

2016; Pg 84

Question 15

Fructose-1,6-bisphosphatase is the rate-determining enzyme for which metabolic process?

Answer 15

Gluconeogenesis

2016; Pg 84

Question 16

5 required cofactors for the pyruvate dehydrogenase complex

Answer 16

TLC for Nancy:
1. Thiamine pyrophosphate (B1)
2. Lipoic Acid
3. CoA (B5, pantothenic acid)
4. FAD (B2, riboflavin)
5. NAD (B3, niacin)
(2016; Pg 87)

Question 17

Increased NAD+/NADH ratio, ADP, and Calcium activates which complex?

Answer 17

Pyruvate dehydrogenase complex

2016; Pg 87

Question 18

Which is active in the fasting state, FBPase-2 (fructose bisphosphatase-2) or PFK-2 (phosphofructokinase-2)?

Answer 18

FBPase-2

2016; Pg 87

Question 19

Where do you find glucokinase?

Answer 19

In liver and beta cells of pancreas.
(Hexokinase is in all other tissues)
(2016; Pg 87)

Question 20

Patient presents with vomiting, rice-water stools, and garlic breath. What co-factor is being inhibited?

Answer 20

Lipoic acid by arsenic

2016; Pg 87

Question 21

X-linked deficiency in which pyruvate is shunted to lactate (via LDH) and alanine (via ALT)

Answer 21

Pyruvate dehydrogenase complex deficiency

2016; Pg 88

Question 22

Treatment for pyruvate dehydrogenase complex deficiency

Answer 22

Increased intake of ketogenic nutrients or lysine and leucine
(2016; Pg 88)

Lysine and Leucine are the onLy pureLy ketogenic amine acids

Question 23

How do NADH electrons from glycolysis enter the mitochondria?

Answer 23

Either via the malate-asparte or glycerol-3-phosphate shuttle
(2016; Pg 89)

Question 24

Oxidative phosphorylation poison that inhibits ATP synthase

Answer 24

Oligomycin

2016; Pg 89

Question 25

Antimycin A is an electron transport inhibitor that blocks which complex?

Answer 25

3 | 2016; Pg 89

Question 26

CO/CN is an electron transport inhibitor that blocks which complex?

Answer 26

4 | 2016; Pg 89

Question 27

Lactating mammary glands, liver, adrenal cortex, and RBCs are sites for what pathway?

Answer 27

``` HMP shunt (pentose phosphate pathway) (2016; Pg 90) ```

Question 28

Heinz bodies (denatured hemoglobin precipitates within RBCs) and bite cells (resulting from phagocytic removal of Heinz bodies) are found in which pathology?

Answer 28

Glucose-6-phosphate dehydrogenase deficiency | 2016; Pg 90

Question 29

Mode of inheritance of disorder in which patient on antituberculosis drugs eating fava beans presents with acute hemolytic anemia

Answer 29

X-linked recessive (Glucose-6-phosphate dehydrogenase deficiency in which decreased NADPH leads to low glutathione and increased free radicals and peroxides) (2016; Pg 90)

Question 30

Pattern of inheritance and symptoms in essential fructosuria

Answer 30

Autosomal recessive, normally a benign condition with fructose appearing in blood and urine (2016; Pg 91)

Question 31

Autosomal recessive deficiency of aldolase B resulting in inhibition of glycogenolysis and gluconeogensists

Answer 31

``` Fructose intolerance (2016; Pg 91) ```

Question 32

Patient recently undertaking a juice fast complains of severe vomiting and yellowing of his skin and sclera. Serum studies indicate hypoglycemia. Patient reports other family members are "allergic" to honey. What disorder should be considered?

Answer 32

``` Fructose intolerance (2016; Pg 91) ```

Question 33

One-month old infant is being treated for E.coli sepsis. Physical exam shows jaundice, infantile cataracts, and failure to thrive. What disorder should be considered?

Answer 33

Classic galactosemia | 2016; Pg 91

Question 34

Classic galactosemia is due to absence of _____.

Answer 34

Galactose-1-phosphate uridyltransderase (autosomal recessive disorder) (2016; Pg 91)

Question 35

Hereditary deficiency of galactokinase

Answer 35

``` Galactokinase deficiency (2016; Pg 91) ```

Question 36

What contributes to the development of cataracts, retinopathy, and peripheral neuropathy in poorly managed diabetics?

Answer 36

Sorbitol accumulation | 2016; Pg 92

Question 37

The conversion of osmotically active galactitol (via aldose reductase) results from...

Answer 37

High levels of serum galactose | 2016; Pg 92

Question 38

Where in the gut does lactase function?

Answer 38

The brush border | 2016; Pg 92

Question 39

What are the three basic amino acids?

Answer 39

Arginine (Arg), Lysine (Lys), and Histidine (His) | 2016; Pg 92