Catch All for First Exam

Question 1

Which hormone requires cyclic AMP in its singlaing mechanism?

Answer 1

glucagon

Question 2

kinases

Answer 2

Add phosphate groups
carry out the phosphorylation reactions by transferring the gamma phosphate of ATP onto hydroxyl groups of various substrates including lipids, sugars or amino acids and is reversed by the corresponding phosphatases.

Question 3

MODY

Answer 3

reduced ability to raise ATP levels

Question 4

Glucagon release does not alter muscle metabolism because of which of the following?

Answer 4

muscle cells lack the glucagon receptor

Question 5

NADH is typically abundnt in mitochondria but lacking in the cytoplasm

Answer 5

True

Malate shuttle helps to transfer NADH from mito to cytoplasm

Question 6

Phosphorylation turns on glycogen phosphoylase

Answer 6

Turns

Question 7

pyruvate to CO2 is the TCA cycle. the first molecule in the TCA cycle is acetyl CoA

Question 8

What are the products of the TCA cycle?

Answer 8

3 NADH
1 FADH
1 GTP
(x2 for each glucose b/c one for each acetyl CoA)

Question 9

In which one of the following enzyme deficiences would there be increased concentration of liver glycogen?

Answer 9

glucose-6-phosphatase deficieny
g-6-pase is a glycenolysis enzyme
g-6-p to glucose

Question 10

UDP-galactose epimerase deficiency

Answer 10

• benign exercretion of a glucose epimer
• increased concentration of galactose-1-P & UDP-galactose

galactose metabolism

Question 11

Gal-1-put deficiency

Answer 11

galactosemia

Question 12

Classic Galactosemia

Answer 12

liver has lots of galactose-1-phosphate
liver cells die because hepatotoxic
decreased albumin -> edema
decreased bile production -> jaundice
fasting hypoglycemia b/c no gluconeogenesis
caloric insufficiency
hish AST and ALT
renal tubular acidosis
enyme: Galactose-1-PUT

Question 13

Aldolase B Deficiency

Answer 13

children avoid fruit or eat skin of fruit
hepatotoxicity renaltoxicity
fructose intolerance
vomit
spit
jaundice

Question 14

glucose-6-P dehydrogenase deficiency

Answer 14

oxidative phase of pentose phosphate pathway
X-Linked Recessive
fava beans intolerance
no sulfa antibiotics
echogenetic
RBC membrane fragility (erythrocytes)
hemolysis
hemolytic anemia

Question 15

glucose-6-phosphatase deficiency

Answer 15

G6PD is used in the first step of glycogenolysis
GSD1a

Question 16

glycogen phosphorylase deficiency

Answer 16

second-wind
rhabdomyolysis (muscle death?)
moderate exercise only
low lactate production

GSDV

Question 17

Does pyruvate kinase deficiency result in lactic acidosis?

Answer 17

NO
PEPP to pyruvate (last step of glycolysis)

Question 18

Hypoxia-induced factor-1 is a key genetic regulator for many genes for glycolytic enzymes

Answer 18

True

Question 19

Deficiency of glycolytic enzymes often result in hemolysis

Question 20

Glycolysis plays a role in fructose metabolism