Part 2 Flashcards

1
Q

How much of the body’s energy needs can be met by amino acid oxidation in the TCA cycle?

A

10-15% of the body’s energy needs.

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2
Q

When do amino acids become a primary source of energy?

A

During starvation, prolonged fasting, or in conditions like diabetes mellitus when carbohydrates are unavailable or cannot be used properly.

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3
Q

What is gluconeogenesis?

A

The process of glucose formation from non-carbohydrate sources.

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4
Q

Which organs primarily perform gluconeogenesis?

A

Liver and kidneys.

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5
Q

Which tissues rely on glucose produced from amino acids during starvation?

A

Brain, red blood cells (RBCs), and other tissues.

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6
Q

What distinguishes glucogenic from ketogenic amino acids?

A

Glucogenic amino acids degrade into pyruvate or TCA cycle intermediates; ketogenic amino acids degrade into Acetyl CoA or Acetoacetyl CoA.

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7
Q

Name four specialized products synthesized from amino acids.

A
  • Porphyrins
  • Neurotransmitters
  • Hormones
  • Purines/pyrimidines
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8
Q

What two molecules combine with glycine to form heme?

A

Succinyl CoA and glycine.

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9
Q

Which amino acids are required for creatine synthesis?

A
  • Glycine
  • Arginine
  • Methionine
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10
Q

What role does glycine play in detoxification?

A

It conjugates with benzoic acid to form hippuric acid for detoxification.

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11
Q

What is the main function of creatine phosphate?

A

To maintain intracellular ATP levels during intense muscular contraction.

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12
Q

How is creatinine used in medical diagnostics?

A

Its levels in serum (along with blood urea) are used as a diagnostic test for kidney function.

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13
Q

Which sulfur-containing amino acids are discussed?

A
  • Methionine
  • Cysteine
  • Cystine
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14
Q

What is the function of S-Adenosylmethionine (SAM)?

A

SAM donates methyl groups to various acceptors and is involved in the synthesis of polyamines like spermine and spermidine.

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15
Q

What conditions are associated with high homocysteine levels?

A
  • Atherosclerosis
  • Osteoporosis
  • Alzheimer’s disease
  • Parkinson’s disease
  • Stroke
  • Cardiovascular diseases
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16
Q

Which vitamins help reduce homocysteine levels?

A
  • Folate
  • Vitamin B6
  • Vitamin B12
17
Q

What enzyme deficiency causes homocystinuria?

A

A deficiency of cystathionine synthase.

18
Q

Name three branched-chain amino acids.

A
  • Valine
  • Leucine
  • Isoleucine
19
Q

What is the primary metabolic disorder associated with branched-chain amino acids?

A

Maple Syrup Urine Disease (MSUD).

20
Q

What enzyme deficiency causes MSUD?

A

A deficiency of α-Keto acid dehydrogenase.

21
Q

What symptoms are associated with MSUD?

A
  • Acidosis
  • Lethargy
  • Convulsions
  • Mental retardation
  • Coma
  • Potentially death within the first year of life
22
Q

How can mild cases of MSUD be treated?

A

Mild cases respond to high doses of thiamine; severe cases may require liver transplantation.

23
Q

Which amino acid is cleaved by arginase to produce urea and ornithine?

A

Arginine.

24
Q

What condition results from defective arginase?

A

Hyperargininemia, an inborn error of arginine metabolism.

25
Q

Which enzyme catalyzes the formation of nitric oxide (NO) from arginine?

A

Nitric oxide synthase (NOS).

26
Q

Name two therapeutic uses of nitric oxide (NO).

A
  • Treatment of angina pectoris
  • Treatment of pulmonary hypertension
27
Q

What condition can result from a deficiency of folic acid in histidine metabolism?

A

Accumulation of FIGLU in urine.

28
Q

What inhibitory neurotransmitter is derived from glutamate?

A

γ-Amino butyrate (GABA).

29
Q

Which vitamin deficiency can lead to reduced GABA levels and convulsions?

A

Vitamin B6 deficiency.

30
Q

What is the function of the Alanine-Pyruvate shuttle?

A

To transport pyruvate as alanine from muscles to the liver for gluconeogenesis.