Cramming 4 Flashcards

1
Q

A valiant knight develops vomiting, garlic odor breath, and rice-water stools after drinking a potion concocted by his arch enemy, the dark wizard. What substance was in this potion and what is its mechanism of action?

A

arsenic; inhibits lipoate, thereby inhibiting glycolysis and the TCA cycle.
(Arsenic can also be found in some electronics, pesticides, herbicides, and contaminated groundwater.)

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1
Q

What enzyme deficiency can cause hemolytic anemia of the newborn? Inheritance pattern?

A

PK; AR

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2
Q

Which are the two purely ketogenic aa’s?

A

Lysine and Leucine

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3
Q

Which enzyme of gluconeogenesis requires GTP? Where is this enzyme found?

A

PEP carboxykinase; cytosol

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4
Q

What reaction in the TCA cycle produces GTP?

A

succinyl CoA –> succinate via succinate thiokinase

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5
Q

What is the difference between odd- and even-chain FA’s w/ regard to gluconeogenesis?

A

OCFAs can participate in gluconeogenesis and ECFAs can’t.

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6
Q

How can odd-chain FAs produce new glucose?

A

Propionyl-CoA->->succinyl-CoA->->OAA

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7
Q

How does the oxidation of FAs produce ATP?

A

β-oxidation yields NADH, FADH2, and acetyl-CoA

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8
Q

What 2 things does the HMP shunt provide?

A

NADPH and ribonucleotides

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9
Q

What processes is NADPH necerssary for?

A

FA and cholesterol synthesis, drug metabolism (glutathione), prevention of oxidative damage (RBCs), and the respiratory burst.

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10
Q

NADPH oxidase deficiency causes _______.

A

Chronic granulomatous disease - phagocytes can ingest microbes but can’t kill them

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11
Q

Pts w/ chronic granulomatous disease are at increased risk for infection w/ _______ species, such as _______ and _______.

A

catalase (+); S. aureus; Aspergillus

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12
Q

Pt. w/ recurrent Candida infections. NΦs turn blue (+ result) on nitroblue tetrazolium test during respiratory burst. What could this pt’s Dx be?

A

MPO deficiency

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13
Q

Which 2 aa’s are essential only during growth?

A

Arg and His

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14
Q

What reaction does ornithine transcarbamoylase (OTC) catalyze?

A

ornithine + carbamoyl phosphate –> citrulline

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15
Q

The formation of which 2 substrates of the urea cycle each use up an ammonia?

A

carbamoyl phosphate and arginosuccinate

16
Q

What is the point of the Cori cycle?

A

To have the liver convert lactate produced by muscle, RBCs back to glucose.

17
Q

What are the treatments for hyperammonemia?

A

limit protein intake, ammonia binders (benzoate and phenylbutyrate), and lactulose

18
Q

How does lactulose combat hyperammonemia?

A

acidifies GI tract to trap ammonium

19
Q

What aa is niacin derived from?

A

Trp

20
Q

What aa is serotonin derived from?

A

Trp

21
Q

What cofactor is necessary to convert Trp to serotonin? What cofactor is necessary to turn Trp into niacin?

A

BH4; B6

22
Q

PKU can be d/t a deficiency of Phe hydroxylase or _______.

A

tetrahydrobiopterin reductase

23
Q

So, BH4 is a required cofactor for the production of what 4 substances?

A

Tyr, DOPA, 5-HT, and NO

24
Q

What aa is converted to NO?

A

Arg