Amino Acid Metabolism Flashcards

1
Q

In what types of cells will you find aminotransferases?

A

Liver, kidney, muscle

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2
Q

Other than the TCA cycle, what pathways are primary consumers of TCA intermediates?

A

Gluconeogenesis

Fatty acid synthesis

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3
Q

What complex is sensitive to ROS, creates succinyl CoA and NADH/FADH2?

A

Alpha-ketoglutarate dehydrogenase

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4
Q

What are the names of vitamins B1, 2, 3, and 5 and what cofactors do they help provide?

A

B1: Thymine, TPP
B2: Riboflavin, FAD+
B3: Niosin, NAD+
B5: Pantothenic acid, CoA

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5
Q

The branched-chain alpha-keto acid dehydrogenase (BCKD) is in the same family as what other dehydrogenase and uses which 5 cofactors?

A
Family = PDC
Cofactors = TPP, Lipoic acid, CoA, NAD+, FAD
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6
Q

Is homocystein good or bad?

A

Bad

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7
Q

What does homocystein accumulation in the kidney cause?

A

Homocystine formation (disulfide bonding)

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8
Q

What is the pathway of Met to Succinyl CoA?

A

Met –> SAM –> SAH –> Homocysteine –(B6, 12, folic acid)–> Succinyl CoA

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9
Q

What causes the defective metabolism of homocystein?

A

Vitamin deficiencies in B6, B12, folic acid

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10
Q

What is hyperhomocysteinemia associated with?

A

Atherosclerotic heart, stroke, eye lens dislocation

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11
Q

What leads to Phenylketonuria and what is a treatment for it?

A
Defects in THB production.
Sapropterin treatment (synthetic THB)
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12
Q

What amino acid is not converted to melanin in albinism?

A

Tyrosine

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13
Q

MAO and COMT breakdown which neurotransmitters?

A

Dopamine: HVA

Norepinephrine/Epinephrine: VMA

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14
Q

What is a pheochromocytoma?

A

Adrenal gland tumor

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15
Q

What does an MAO inhibitor do?

A

Acts as anti-depressant by inhibiting degradation of neurotransmitters in synaptic cleft

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16
Q

How can hyperthyroidism be treated?

A

Agents to block iodination of thyroglobulin and decrease T4/T3 production

17
Q

T/F: Glutathione acts as a neurotransmitter.

A

False, antioxidant

18
Q

What affect does increased levels of acetaminophen have on glutathione levels?

A

Depletes them because they bind together

19
Q

Elevated gamma-glutamyl transpeptidase (GGT) is a marker for what?

A

Hepatobiliary dysfunction

20
Q

What do nitrodilators do to BP?

A

Decrease BP and relax smooth muscle

21
Q

Explain nNOS.

A

NOSI, on neuronal cells

Messenger and modulator of neurotransmitter release

22
Q

Explain iNOS.

A

NOSII, Phagocytic inducible isoform,

Fights infections in macrophages

23
Q

Explain eNOS.

A

NOSIII, on Endothelial cells,

Prevents vascular smooth muscle contraction and platelet aggregation by increasing Ca2+ and intracellular cGMP

24
Q

Serum cardioselective creatine kinase is a marker for what and peaks when?

A

M.I.

Peaks 10-24 hrs post M.I.

25
Q

Carbamoyl phosphate synthetase II is the enzyme that catalyzes the first step of de novo synthesis of what?

A

Pyrimidines

26
Q

What stimulates and inhibits carbamoyl phosphate synthetase II?

A

Stimulates: PRPP
Inhibits: UTP

27
Q

What autosomal recessive defect leads to accumulation of orotic acid and orotic aciduria?

A

UMP synthase

28
Q

Is urea increased/decrease for high protein/carb diets?

A

Protein - Increased urea

Carb - Decreased urea

29
Q

Which amino acids are strictly ketogenic?

A

Leu, Lys

30
Q

Which amino acids are both ketogenic and glucogenic?

A

Ile, Trp, Phe, Tyr, Thr

31
Q

What test is used for nitrogen balance and what is the normal range?

A

Blood Urea Nitrogen (BUN)

3.0-8.2 mg/dL

32
Q

Which aminotransferase is found only in the cytoplasm and which is found in both the cytoplasm and mitochondria?

A

Cytoplasm: ALT
Both: AST

33
Q

Which is a more specific diagnosis of liver disease, ALT or AST?

A

ALT