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Uworld > Test 1-3: Biochemistry > Flashcards

Test 1-3: Biochemistry Flashcards

1
Q

Synchronization of glycogen degradation with skeletal muscles contraction occurs due to what

A

release of sarcoplasmic calcium following neuromuscular stimulation

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2
Q

Increased intracellular calcium in skeletal muscles activates what

A

phosphorylase kinase: stimulating glycogen phosphorylase to increase glycogenolysis

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3
Q

Glycogen is broken down by what enzyme

A

glycogen phosphorylase

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4
Q

What enzyme is responsible for phosphorylation of glycogen phosphorylase

A

Phosphorylase kinase

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5
Q

what catalyzes dephosophrylation of glycogen phosphorylase

A

phosphoprotein phosphatase

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6
Q

How is phosphorylase kinase activated in the liver?

A
  1. Epinephrine and glucagon

2. increases [cAMP]

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7
Q

What are 2 ways skeletal muscles activates phosphorylase kinase

A
  1. Epinephrine-induced increase in cAMP

2. Increase intracellular calcium

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8
Q

Release of sarcoplasmic calcium allows for the synchronization of what?

A
  1. skeletal muscle contraction

2. glycogen breakdown

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9
Q

What can be beneficial in the treatment of measles infections by reducing comorbidites, recovery time, and length of hospital stays?

A

Vitamin A

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10
Q

Pyridoxine

A

B6

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11
Q

Cobalamin

A

Vitamin B12

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12
Q

Most common urea cycle disorder

A

Ornithine transcarbamylase deficiency

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13
Q

how is ornithine transcarbamylase deficiency inherited

A

X-linked recessive

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14
Q

What is found in the the system of an ornithine transcarbamylase deficiency patient? they have symptoms of what?

A
  • increased orotic acid in blood and urine
  • decrease BUN
  • hyperammonemia
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15
Q

Why is there increased orotic acid in blood and urine for ornithine transcarbamylase

A

excess carbamoyl phosphate is converted to orotic acid

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16
Q

what is the rate limiting enzyme in the urea cycle

A

Carbamoyl phosphate synthetase I

- convertes bicarb, ammonia and ATP to carbamoyl phosphate

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17
Q

What acts as the regulator of the urea cycle through activation of carbamoyl phosphate synthetase I

A

N-acetylglutamate

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18
Q

What are some symptoms for hyperammonemia

A

episodes of vomiting, and confusion/coma

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19
Q

tachypnea

A

rapid breathing

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20
Q

What is the problem in orotic aciduria

A

uridine monophosphate synthetase deficiency

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21
Q

Which one has megaloblastic anemia: ornithine transcarbamylase deficiency or orotic aciduria

A

orotic aciduria

22
Q

Which one has hyper ammonia: ornithine transcarbamylase deficiency or orotic aciduira

A

ornithine transcarbamylase deficiency

23
Q

Bloom syndrome: what is the problem

A

mutations in BLM gene encoding DNA helicase

24
Q

How do Bloom syndrome patients present

A
  • growth retardation
  • facial anomalies
  • photosenstive skin rash
  • immunodeficiency
25
Thiamine is a cofactor for what 3 things
1. pyruvate dehydrogenase 2. alpha-ketoglutarate dehydrogenase 3. transketolase
26
Administration of glucose to thiamine deficient patients can results in
Wernicke encephalopathy due to increased thiamine demand
27
Metabolism of alcohol increases what ratio
NADH/NAD+
28
In setting of Wernicke encephalopathy, thiamine-dependent enzymes are especially affected due to the fact of what factors
1. NAD+ ( required for TCA) | 2. thiamine
29
In addition to lowering blood glucose, insulin increases glycogen synthesis in hepatocytes by activating what enzyme
Protein phosphatase
30
What receptor can activates protein phosphatase
Tyrosine kinase dimer receptor, with insulin
31
Southern blotting is used to detect
DNA mutations
32
Familial hypercholesterolemia is defective in what
Absent or defective LDL receptors
33
Familial hypercholesterolemia is inherited how
autosomal dominant
34
What is telomerase? What does it to
- Reverse transcriptase ( RNA-dependent DNA polymerase) | - lengthens telomeres by adding TTAGGG repeats to 3' of chromosomes
35
What type of cells have long telomeres
Stem cells | - high telomerase activity
36
What serves as a source of glucose during fasting and as an energy store that can be mobilized quickly during strenuous muscle
glycogen
37
What are the glycogen storage disease
Very Poor Carbohydrate Metabolism - Von Gierke - Pompe - Cori - McArdle
38
Who is someone with muscle cramps, myoglobinuria ( red urine) with strenuous exercise, and arrhythmia form electrolyte abnormalities
McArdle disease
39
What enzyme is deficient in McArdle disease
- skeletal muscle glycogen phosphorylase | - Myophosphorylase
40
Increased frequency in patients with activating mutations involving Phosphoribosyl pyrophosphate syntheses leads to what
gout
41
What is the first line therapy for treating acute gouty arthritis
NSAIDS | - inhibits neutrophils
42
What is the second lune of therapy for acute gout
Colchicine
43
MOA for Colchicine
- binds ands stabilizes tubulin to inhibit microtubule polymerization - impairing neutrophil chemotaxis and degranulation
44
What are symptoms for dry Beriber
- symmetrical peripheral neuropathy | - polyneuritis
45
What are symptoms for wet beriberi
- high output cardiac failure (cardiomyopathy) | - edema
46
hemorrhage and necrosis in the mammillary bodies and periaqueductal gray matter are found in what problem
Wernicke-Korsakaff syndrome
47
Activity of what can diagnosed thiamine deficiency
- baseline erythrocyte transketolase activity is low | - but then increases after addition of thiamine pyrophosphate
48
Protoporphyrin is a precursor for what
heme
49
What is the rate-limiting step in pentose phosphate pathway
Erythrocyte glucose-6-phosphate dehydrogenase
50
When do you see methylmalonic acid levels increased
Vitamin B12 deficiency
51
55-year old farmer: pupils are symmetric, 2mm, and reactive to light. Eyes are tearing considerably. Scattered wheezes bilaterally on lung auscultation . Skin is clammy and he is sweating profusely? toxicity ?
Organophosphates | - used in pesticides in agriculture
52
MOA for organophosphates
- inhibit breakdown of acetylcholine

Uworld (63 decks)

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