Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

First Aid STEP 1 - Biochemistry > Biochemistry- Metabolism > Flashcards

Biochemistry- Metabolism Flashcards

1
Q

What metabolic functions occur in the mitochondria?

A

fatty acid oxidation (beta-oxidation)
acetyl-CoA production
TCA cycle
oxidative phosphorylation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What metabolic functions occur in the cytoplasm?

A 
glycolysis
fatty acid synthesis
cholesterol synthesis
steroid synthesis (SER)
protein synthesis (RER)
HMP shunt
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What metabolic functions occur in both the cytoplasm and mitochondria?

A

Heme synthesis
Gluconeogenesis
Urea cycle

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the function of a kinase?

A

Uses ATP to add a phosphate group onto a substrate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the function of a phosphorylase?

A

Adds a phosphate group onto a substrate WITHOUT using ATP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the function of a phosphatase?

A

Removes a phosphate group

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the function of a dehydrogenase?

A

catalyzes oxidation-reduction reactions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the function of a hydroxylase?

A

adds a hydroxyl group onto a substrate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the function of a carboxylase?

A

transfers CO2 groups with the help of biotin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the function of a mutate?

A

relocates a functional group within a molecule

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How many ATP does aerobic glycolysis produce?

A

32

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

How many ATP does anaerobic glycolysis produce?

A

2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How many ATP does arsenic cause glycolysis to produce?

A

0

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What type of processes is NAD+ generally used in?

A

catabolic processes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What type of processes is NADPH generally used in?

A

anabolic processes (steroid and fatty acid synthesis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

NADPH is also used in what other functions of the cell?

A

anabolic processes
respiratory burst
cytochrome P-450 system
glutathione reductase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Where is hexokinase located and what are some characteristics about the enzyme?

A

Most tissues

higher affinity (lower Km)
higher capacity (lower Vmax)
NOT induced by insulin
Feedback-inhibited by glucose-6-phosphate
NO gene mutation assoc. w/ maturity-onset diabetes of the young (MODY)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Where is glucokinase located and what are some characteristics about the enzyme?

A

Liver, beta-cells of pancreas

lower affinity (higher Km)
lower capacity (higher Vmax)
Induced by insulin
Feedback is NOT induced by glucose-6-phosphate
Gene mutation associated with maturity-onset of diabetes of the young (MODY)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

How many ATP are produced through glycolysis?

A

2 ATP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

FBPase-2 an PFK-2 are the same bifunctional enzyme who function is reversed by phosphorylation by what protein?

A

protein kinase A

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is the order of substrates in the TCA cycle?

A 
Citrate
Isocitrate
alpha-ketoglutarate
Succinyl-Coa
Succinate
Fumarate
Malate
Oxaloacetate

Citrate Is Kreb’s Starting Substrate For Making Oxaloacetate!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What are some electron transport inhibitors?

A

Rotenone (Complex I)
Cyanide (Complex IV)
Antimycin A (Complex III)
CO (Complex IV)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is an ATP synthase inhibitor?

A

Oligomycin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What are some uncoupling agents?

A

2,4- dinitrophenol
aspirin
thermogenin in brown fat

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What are the enzymes necessary for gluconeogenesis?

A

Pyruvate carboxylase
Phosphoenolpyruvate carboxykinase
Fructose-1,6-biphosphatase
Glucose-6-phosphatase

Pathway Produces Fresh Glucose

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What organ does gluconeogenesis primarily occur in?

A

liver

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What does propionyl-CoA form from?

A

odd chain fatty acids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Where in the TCA cycle can propionyl-CoA enter?

A

Succinyl-CoA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What are major products of the HMP shunt and what are these products used for in the body?

A

NADPH –> glutathione reduction inside RBCs, FA and cholesterol biosynthesis

Ribose –> nucleotide synthesis and glycolytic intermediates

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Where does the HMP shunt occur?

A

lactating mammary glands
liver
adrenal cortex (sites for FA or steroid synthesis)
RBCs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What is the purpose of the respiratory burst (oxidative burst)?

A

Plays an important role in the immune response (both creation and neutralization with ROS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What type of infections are patients with chronic granulomatous disease at an increase risk for and why?

A

Catalase + microorganisms because these organisms can neutralize their own H2O2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What is the most common human enzyme deficiency?

A

Glucose-6-phosphate dehydrogenase deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What population is G6PD def. more common in?

A

African Americans

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

What can precipitate hemolytic anemia in individuals with G6PD deficiency?

A 
fava beans
sulfonamides
primaquine
anti-TB drugs
infections
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

What type of inheritance is G6PD deficiency?

A

X-linked Recessive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

What symptoms are seen with essential fructosuria? Is it a serious condition?

A

Fructose in the blood and urine

Benign condition

38
Q

What inheritance pattern is seen in both essential fructosuria and fructose intolerance?

A

Autosomal recessive

39
Q

What is the sequelae of fructose intolerance?

A

fructose-1-phosphate accumulates –> decrease in available phosphate –> inhibition of glycogenolysis and gluconeogenesis

40
Q

What are symptoms of fructose intolerance?

A

hypoglycemia
jaundice
cirrhosis
vomiting

41
Q

What is the treatment for fructose intolerance?

A

decrease intake of both fructose and sucrose

42
Q

What substrate accumulates in galactokinase deficiency?

A

galactitol

43
Q

What are symptoms of galactokinase deficiency?

A

galactose in blood and urine
infantile cataracts
may present as failure to track objects or to develop a social smile

44
Q

What enzyme is deficient in classic galactosemia?

A

galactose-1-phosphate uridyltransferase

45
Q

What are symptoms of classic galactosemia?

A 
failure to thrive
jaundice
hepatomegaly
infantile cataracts
intellectual disability
46
Q

What is the treatment for classic galactosemia?

A

exclude galactose and lactose (galactose + glucose) from the diet

47
Q

What is the pathogenesis of diabetes symptoms due to sorbitol metabolism?

A

chronic hyperglycemia –> increase glucose converted to sortbitol –> increase sorbitol accumulation –> osmotic damage –> peripheral neuropathy, cataracts, and retinopathy

48
Q

What are findings associated with lactase deficiency?

A

bloating, cramps, flatulence, and osmotic diarrhea

49
Q

What is the treatment for lactase deficiency?

A

avoid dairy products

add lactase pills

50
Q

What are the essential amino acids?

A 
Phe
Val
Thr
Trp
Ile
Met
His
Arg
Leu
Lys

PVT TIM HALL

51
Q

What are the acidic amino acids?

A

Asp. and Glu

52
Q

What are the basic amino acids?

A

Arg., Lys, and His

53
Q

Which amino acids are increased in histones?

A

Arg and Lys

54
Q

What is the function of the urea cycle?

A

Excess nitrogen (NH3) generated by amino acid metabolism is converted to urea to be excreted by the kidneys

55
Q

What is the pneumonic to remember the order for the urea cycle?

A

Ordinarily, Careless Crappers Are Also Frivolous About Urination

56
Q

What are causes of hyperammonemia?

A

acquired (liver disease)

hereditary (urea cycle enzyme deficiencies)

57
Q

What condition is caused by N-acetylglutamate deficiency?

A

hyperammonemia

58
Q

What finding would indicate an N-acetylglutamate deficiency vs. a carbamoyl phosphate synthase I deficiency?

A

increased ornithine with normal urea cycle enzymes suggests hereditary N-acetylglutamate deficiency

59
Q

What is the amino acid derivative of NE and epi?

A

phenylalanine

60
Q

What is the amino acid derivative of NAD+/NADP+?

A

tryptophan

61
Q

What is the amino acid derivative of melatonin?

A

tryptophan

62
Q

What is the amino acid derivative of histamine?

A

histidine

63
Q

What is the amino acid derivative of heme?

A

glycine

64
Q

What is the amino acid derivative of GABA and glutathione?

A

glutamate

65
Q

What is the amino acid derivative of creatine, urea, and NO?

A

arginine

66
Q

What disease is caused by a deficiency in phenylalanine hydroxyls?

A

PKU

67
Q

What disease is caused by a deficiency in tyrosinase?

A

albinism

68
Q

What disease is caused by a deficiency in homogentisate oxidase?

A

alkaptouria

69
Q

What is the treatment for homocystinuria due to cystathione synthase deficiency?

A

decrease methionine
increase cysteine
increase B12 and folate in diet

70
Q

What is the treatment for homocystinuria due to a decreased affinity of cystathione synthase for B6?

A

REALLY increase B6 in diet

increase cysteine in diet

71
Q

What is the treatment for homocystinuria due to homocysteine methyltransferase deficiency?

A

increase methionine in diet

72
Q

What are the findings for homocystinuria?

A 
REALLY increased homocysteine in urine
intellectual disability
osteoporosis
atherosclerosis
tall stature
kyphosis
lens subluxation (down and inward)
thrombosis
73
Q

What is the cause of cystinuria?

A

defect in transport in PCT in kidney and intestinal cells

74
Q

What amino acids or substrates does the defected transporter in cystinuria usually transport?

A

COLA

Cysteine
Ornithine
Lysine
Arginine

75
Q

What test is diagnostic for cystinuria?

A

urinary cyanide-nitroprusside test

76
Q

What mode of inheritance is cystinuria?

A

AR

77
Q

What is the enzyme deficiency in Maple Syrup Urine Disease?

A

decrease in alpha-ketoacid dehydrogenase (B1)

78
Q

What amino acids accumulate in Maple Syrup Urine Disease?

A

isoleucine
leucine
valine

79
Q

What is the mode of inheritance for Maple Syrup Urine Disease?

A

AR

80
Q

What are the physical findings in Maple Syrup Urine Disease?

A

severe intellectual disability
severe CNS defects
urine smells like maple syrup/burnt sugar
death

81
Q

What is the treatment for Maple Syrup Urine Disease?

A

restrict leucine, isoleucine, and valine in diet

thiamine supplementation

82
Q

What enzyme cleaves glucose - 1- phosphate residues off branched glycogen?

A

glycogen phosphorylase

83
Q

What is the enzyme deficient in Von Gierke Disease (type I)?

A

glucose-6-phosphate

84
Q

What is the treatment for Von Gierke Disease?

A

frequent oral glucose/cornstarch

avoidance of fructose and galactose

85
Q

What is the enzyme deficient in Pompe Disease (type II)?

A

lysosomal alpha - 1, 4- glucosidase (acid maltase)

86
Q

What are the findings in Pompe Disease?

A

cardiomyopathy and systemic findings leading to early death

hypotonia

87
Q

What is the enzyme deficient in Cori Disease (type III)?

A

debranching enzyme (alpha-1,6-glucosidase)

88
Q

What are the findings in Cori Disease?

A

milder form of type I (Von Gierke Disease) with NORMAL blood lactate levels

89
Q

What is the enzyme deficiency in McArdle disease (type V)?

A

skeletal muscle glycogen phosphorylase

90
Q

What are the findings in McArdle disease?

A

increased glycogen in muscle but cannot break it down –> MUSCLE CRAMPS, MYOGLOBINURIA, and ARRHYTHMIA (d/t electrolyte imbalances)

First Aid STEP 1 - Biochemistry (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions