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BIOCHEMISTRY LEC > AMINO ACIDS AND PROTEINS > Flashcards

AMINO ACIDS AND PROTEINS Flashcards

1
Q

Polymers of amino acids

A

Proteins

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2
Q

Number of naturally occurring amino acids

A

300

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3
Q

number of constitute the monomer units of proteins

A

20

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4
Q

Definition of Amino Acids

A

▪ Building blocks of proteins
▪ Determine the biologic activity of the protein
▪ Growth, repair, and maintenance of all cells
▪ Synthesis of body proteins
▪ Synthesis of non-protein nitrogen-containing compounds

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5
Q

Classification of Amino Acids

A
  1. Nutritional Perspective
  2. Metabolic Fate
  3. Polarity
  4. Structure of the Side Chain
  5. pH
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6
Q

number of nutritionally non—essential – produced during glycolysis and of the citric acid cycle

A

12

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7
Q

Provides all essential amino acids

A

Complete protein

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8
Q

Deficient in one or more of the essential amino acids

A

Incomplete proteins

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9
Q

Missing: Rice, corn, and wheat

A

lysine

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10
Q

Missing: threonine

A

Rice

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11
Q

Missing: tryptophan

A

corn

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12
Q

Deficient: methionine

A

Beans, peas, and other legumes

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13
Q

Body does not synthesize them and must be supplied through the diet

A

Essential or indispensable amino acids

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14
Q

_________ is produced from phenylalanine

A

Tyrosine

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15
Q

Tyrosine is produced from _________

A

phenylalanine

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16
Q

Conditionally essential and supplied exogenously to specific populations

A

Semi–essential amino acids

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17
Q

________ and ________ can be synthesized by adults and not by growing
children

A

arginine and histidine

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18
Q

body can synthesize _________ amino acids to meet the biological needs

A

10

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19
Q

Essential Amino Acids

A

Arginine
Histidine
Methionine
Isoleucine
Phenylalanine
Threonine
Tryptophan
Leucine
Lysine
Valine

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20
Q

Non-Essential Amino Acids

A

Alanine
Asparagine
Aspartic Acid
Glutamic Acid
Glycine
Glutamine
Proline
Cysteine
Serine
Tyrosine

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21
Q

Has both anabolic and catabolic processes

A

amphibolic

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22
Q

_________ amino acids can not be stored and thus are catabolized (metabolized)

A

Excess

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23
Q

Generate precursors of glucose or glycogen

A

Glucogenic amino acids

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24
Q

Glucogenic amino acids Precursors:

A

pyruvate or a citric acid cycle intermediate

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25
Q

Alanine deaminated to _________

A

pyruvate

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26
Q

_________ deaminated to pyruvate

A

Alanine

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27
Q

Arginine converted to __________

A

α-ketoglutarate

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28
Q

__________ converted to α-ketoglutarate

A

Arginine

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29
Q

Aspartate converted to _________

A

oxaloacetate

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30
Q

_________ converted to oxaloacetate

A

Aspartate

31
Q

Can be converted into fat (fat producing) or Acetyl CoA

A

Ketogenic amino acids

32
Q

_______ can be synthesized from amino acids

A

Fat

33
Q

Ketogenic amino acids Generate ________

A

ketone bodies

34
Q

Generate precursors of both glucose and fats

A

Glucogenic and Ketogenic amino acids

35
Q

Class of inherited errors of metabolism where There is an enzyme defect that inhibits the body’s ability to metabolize certain amino acids

A

Aminoacidopathies

36
Q

defined the concept of inborn errors of metabolism

A

1909 Garrod

37
Q

concept of inborn errors of metabolism:

A

▪ Blocks in specific metabolic pathways result from defects in particular enzymes
▪Defective or absent protein is an enzyme

38
Q

Probable reasons of aminoacidopathies:

A

A. Activity of a specific enzyme in the metabolic pathway
B. Membrane transport system for amino acids

39
Q

Aminoacidopathies

A

Phenylketonuria PKU
Tyrosinemia

Maple Syrup Urine Disease MSUD

Alkaptonuria (Black urine disease)

Cystinuria (Hartnup’s Disease)

40
Q

Phenylalanine hydroxylase (PAH) deficiency

A

Phenylketonuria PKU

41
Q

First newborn screening test introduced

A

(early 1960s)

42
Q

an absence of activity of the enzyme phenylalanine hydroxylase (PAH), which catalyzes the conversion of phenylalanine to tyrosine

A

Phenylketonuria Mechanism

43
Q

Chronically high levels of phenylalanine results to

A

brain problems

44
Q

Hyperphenylalaninemia cases that are not the
result of the lack of the PAH enzyme

A

Deficiency in the enzymes for regeneration and
synthesis of tetrahydrobiopterin (BH4)

45
Q

cofactor for enzymatic hydroxylation of the
aromatic amino acids

A

BH4

46
Q

Phenylketonuria Absence of enzyme:

A

Phenylalanine levels = >1,200 μmol/L
Newborn: upper limit of normal = 120 μmol/L (2 mg/dL)

Metabolites: blood and urine (characteristic musty odor)

47
Q

Women with PKU (untreated during pregnancy):

A

microcephalic and mentally retarded babies

48
Q

(Phenylketonuria) Insufficient synthesis of _________

A

serotonin

49
Q

is the pigment synthesized from tyrosine by tyrosinase:

A

Melanin

50
Q

Phenylketonuria Management

A

Sapropterin dihydrochloride (Kuvan®)

Reduce phenylalanine levels by increasing the activity of the PAH enzyme

51
Q

Phenylketonuria Management 2

A

Pegvaliase-PQPZ (Palynziq®)

First FDA-approved enzyme substitution therapy as of 2018, substitutes a PEGylated version of the enzyme phenylalanine ammonia lyase for the deficient PAH enzyme

52
Q

Phenylketonuria Management 3

A

Dietary restriction of phenylalanine with tyrosine supplementation

53
Q

Characterized by the excretion of tyrosine and tyrosine catabolites in urine

A

Tyrosinemia

54
Q

Tyrosinemia Most severe form
Low levels of the enzyme fumarylacetoacetate hydrolase

A

Type I

55
Q

Type I Tyrosinemia

A

Leads to liver and kidney failure, problems affecting the nervous system, and an increased risk of cirrhosis or liver cancer

56
Q

Type I Tyrosinemia Management 1

A

Nitisinone (also known as NTBC)

In act on tyrosine metabolism upstream of the defect to prevent the production of these metabolites.

57
Q

Type I Tyrosinemia Management

A

Diet

prescribed a low-phenylalanine, low tyrosine diet

58
Q

AKA Richner-Hanhart syndrome
deficiency of the enzyme tyrosine aminotransferase:

A

Type II Tyrosinemia

59
Q

Type II Tyrosinemia Management

A

No cure for this condition

Some of the symptoms may be managed with a diet:

60
Q

Rare disorder (only a few cases have been reported)
Deficiency of the enzyme 4- hydroxyphenylpyruvate dioxygenase:

A

Type III Tyrosinemia

61
Q

Type III Tyrosinemia Clinical Presentation

A

Mild mental retardation, seizures, and periodic loss of balance and coordination

62
Q

Type III Tyrosinemia Management

A

▪ Follow a phenylalanine- and tyrosine-restricted diet
▪ No available treatment yet

63
Q

Defective enzyme: homogentisate oxidase in tyrosine metabolism

Homogentisate accumulates in tissues and blood → excreted into urine

A

Alkaptonuria

64
Q

Alkaptonuria Clinical Presentation

A

Ochronosis:, Arthritis, Signs of aortic or mitral valvulitis

65
Q

Alkaptonuria Management

A

➢Ascorbic Acid
➢ Nitisinone

65
Q

Absence or greatly reduced activity of the enzyme branched-chain α-ketoacid dehydrogenase

→ blocking normal metabolism of leucine, isoleucine, and valine

A

Maple Syrup Urine Disease (MSUD)

66
Q

Degradation of the branched-chain amino acids:

A

➢ Valine → propionyl CoA
➢ Isoleucine → propionyl CoA and acetyl CoA
➢ Leucine → acetoacetate and acetyl CoA

67
Q

Maple Syrup Urine Disease (MSUD) Clinical Presentation

A

Characteristic maple syrup or burnt
sugar odor: urine, breath, and skin.

68
Q

Maple Syrup Urine Disease (MSUD) Management

A

▪Thiamine
▪ Dietary restriction of branched-chain AA

69
Q

Amino acid transport disorder

Defective transport of cystine and basic amino acids across renal tubule and the small
intestine.

A

Cystinuria

70
Q

Cystinuria Clinical Presentation

A

▪ Formationof cystine stones in the kidney, due to its low solubility
▪ Chronic urinary tract infections
▪ Hematuria and dysuria

71
Q

Cystinuria Management

A

▪ Hydration
▪ Urinary Alkalinizers
▪ Chelating Agents
▪Crystal Growth Inhibitors

72
Q

to decrease urinary cystine concentration

A

Hyperdiuresis

73
Q

Urinary Alkalinizers

A

➢ Potassium Citrate, Acetazolamide

BIOCHEMISTRY LEC (6 decks)

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