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Biochem 1 > Branched Chain Amino Acids, Aromatic Amino Acids etc > Flashcards

Branched Chain Amino Acids, Aromatic Amino Acids etc Flashcards

1
Q

What is a glucogenic AA?

A

AA that can be converted to glucose

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2
Q

What are the glucogenic AA’s

A

All AA’s except leucine and lysine

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3
Q

What is a ketogenic AA?

A

AA that can make ketone bodies (acetyl Co-A)

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4
Q

What are the ketogenic AA’s?

A

Leucine and lysine

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5
Q

What are glucogenic AAs that can also produce ketone bodies?

A 
Phenylalanine
Isoleucine
Threonine
Tryptophan
Tyrosine
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6
Q

What are the branch chain AAs?

A

Valine
Isoleucine
Leucine

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7
Q

What are the steps involved in metabolism of branch chain AAs?

A
  1. Transamination

2. Oxidative carboxylation by an a-keto acid dehydrogenase

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8
Q

What is maple syrup urine disease?

A

defect in branch chain a-keto acid dehydrogenase complex

Therefore there is an accumulation of an a-keto acid

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9
Q

What are the characteristics of maple syrup urine disease?

A
  • odor of urine is like maple syrup

- failure to thrive, developmental delay (b/c it affects brain tissue)

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10
Q

What is the therapy for maple syrup urine disease?

A

low protein diet, low in branched-chain AAs

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11
Q

What is can serine be synthesized from?

A

glucose (glycolysis intermediate)

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12
Q

What are the major sites of serine synthesis?

A

liver and kidney

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13
Q

What is one purpose of serine?

A

Can be used as an energy source by tissues

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14
Q

What can glycine be synthesized from

A

serine (also threonine which is minor)

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15
Q

What does the pathway for the biosynthesis of glycine involve?

A

folic acid (CH2 acceptor) and pyridoxal phosphate

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16
Q

What can glycine be converted to?

A

glyoxylate

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17
Q

What are some molecules that glycine is a precursor of?

A 
Creatine
Heme
Glutathione
purine nucleotides
glycochenodeoxycholate (bile salts)
18
Q

Where does synthesis of creatine begin?

A

kidney

19
Q

Where does synthesis of creatine ends?

A

liver

20
Q

Some partially synthesized creatine goes to the liver, where does this creatine go?

A

to other tissues eg heart, skeletal muscle, brain

21
Q

What is one function of creatine?

A

source of high energy phosphate to replenish ATP

22
Q

How is creatinine formed?

A

non-enzymatically
irreversibly
spontaneously
rate of formation is proportional to muscle mass

23
Q

How is creatinine excreted?

A

in urine

24
Q

What does creatinine levels in the urine tells us?

A

kidney function

muscle wasting

25
Q

Maple syrup disease leads to accumulation of:

A. Glucose and fructose in the blood and urine
B. Valine and leucine in the blood and urine
C. Glycine in the blood and urine
D. Alpha-keto acids in the blood and urine

A

D

26
Q

Glutamate is a precursor or product of which AAs?

A

Histidine
Glutamine
Proline
Arginine

27
Q

What is GABA?

A

an inhibitory neurotransmitter

28
Q

What is the precursor of GABA

A

Glutamate

29
Q

What is one thing synthesis of GABA requires

A

Pyridoxal phosphate PLP

30
Q

What can affect the formation (failure to form) of GABA

A

low levels of vit B6

31
Q

How can ornithine form outrescine

A

by decarboxylating ornithine

32
Q

How is NO derived?

A

From the metabolism of arginine

33
Q

What is the precursor of tyrosine?

A

Phenylalanine

It is hydroxylated to form tyrosine

34
Q

What does the formation of tyrosine from phenylalanine requires?

A

tetrahydrobiopterin (BH4, synthesized in our bodies from GTP)

35
Q

What defect causes PKU?

A

defect in phenylalanine hydroxylase (phenylalanine cant be hydroxylated to form tyrosine)

36
Q

What defect causes Alkaptonuria?

A

defect in homogentisate oxidase, which causes buildup in homogentistate which darkens urine

37
Q

What defect causes Albinism?

A

defect in tyrosinase, inability to synthesize melanin pigment
defect in tyrosine metabolism in melanocytes

38
Q

Phenylalanine/tyrosine are precursors for

A

Melanins (pigment molecules)

Neurotransmitters (dopamine norepinephrine, epinephrine)

39
Q

Tryptophan is a precursor for:

A

serotonin (neurotransmitter)

melatonin (circadian rhythms)

40
Q

Classical Phenylketonuria (PKU) results from a defect in phenylalanine hydroxylase. If untreated, this leads to:

A. accumulation of tyrosine in the urine
B. Accumulation of phenylalanine in the urine
C. Accumulation of acetone in the urine
D. Accumulation of phenylacetate in the urine

A

D

Biochem 1 (22 decks)

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