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Proteins > Amino Acid Metabolism > Flashcards

Amino Acid Metabolism Flashcards

1
Q

Name an example of an endopeptidase (3)

A

Pepsin from pepsinogen

Trypsin

Chymotrypsin

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2
Q

What does pepsin cleave

Trypsin

Chymotrypsin

A

L/F/W/Y

K/R

F/W/Y

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3
Q

How do endopeptidases cleave

A

Non terminal part of protein

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4
Q

What do expetidases cleave

A

Terminal end

Aminopeptidase (amino end)
Carboxypeptidase (Carboxyl end)

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5
Q

All aminotransferases have what

A

PLP

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6
Q

What is PLP derived from

A

Vit.B6

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7
Q

Glutamate dehydrogenase is found where

A

Mitochondria

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8
Q

What’s formed in the presence of NH4 + glutamate

A

Glutamine

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9
Q

What is considered the carrier of ammonia

A

Alanine

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10
Q

What is produced from exercise

A

Ammonia —> glutamate

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11
Q

What does the glutamate form with when produced in muscles

A

Forms with pyruvate to form alanine

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12
Q

Alanine combines with what in the liver to form pyruvate + glutamate

A

a-ketogluterate

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13
Q

What can cross the brain barrier which is toxic

What does it react with leading to?

A

Ammonia

Glutamate dehydrogenase

Lack of GABA

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14
Q

Why is ammonia stored in mitochondria

A

Compartmentalised so free NH4 cannot react

No increase in pH

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15
Q

Draw the urea cycle

A

-

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16
Q

What does the urea cycle produce

A

Fumerate (AA)

Ornithine (FA)

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17
Q

What promotes NAG synthase

A

Arginine

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18
Q

What does NAGS promote

A

Cps-1

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19
Q

What does cps-1 converge

A

NH4–> Carbomoyl phosphate

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20
Q

Cps-1 deficiency

What does it lead to

A

Type 1 hyperammonaemia

NH4 build up =toxicity

Retardation

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21
Q

How can we treat type 1
hyperammonaemia

Why

A

Arginine

Stimulates NAG synthase to stimulate any CPS-1

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22
Q

OTC deficiency

A

Type 2 hyperammonaemia

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23
Q

What does type 2 hyperammonaemia lead to

A

Increase ammonia /orotic Acid /glutamine in blood

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24
Q

What causes type 2 hyperammonaemia

A

X-linked genetic defect

25
Q

How can we treat type 2 hyperammonaemia

A

High carbs / low protein(low AA+NH4)

Benzoic Acid for ammonia detoxification

26
Q

Arginosuccinate synthase deficiency

What causes it

Treatment

A

Citrullinemia

Autosomal recessive

Arginine enhances citrulline excretion

27
Q

Arginosuccinic aciduria is caused by what?

How do we treat it

A

Arginosuccinase defiency

(Arginosuccinate —> arginine +fumerate)

Arginine promotes nitrogen excretion

28
Q

What causes arginosuccinic aciduria

What does it lead to for symptoms

A

Arginosuccinase deficiency

Vomiting

29
Q

Hyperargininaemia

How do we treat it

A

Arginase deficiency
Increased ammonia
Retardation
Rare autosomal recessive

Low protein
Low arginine

30
Q

How do we treat hyperargininaemia

A

Low protein diet

Restrict arginine intake

31
Q

What can be used as treatments for urea disorders (2)

A

Benzoate

Phenylacetate

32
Q

How do benzoate / phenylacetate work

A

Converted into CoA thioesters

Combine with glutamine +glycine

Bind to ammonia

Excreted in urine

33
Q

Maple syrup urine disease can’t break down what 3 AA

A

Valine

Isoleucine

Leucine

Chained AA

34
Q

What are maple syrup urine patients lacking

What causes it

A

Lacking BCK dehydrogenase

Autosomal recessive 1 in 150,000

35
Q

What causes the smell of maple syrup

A

Sotolone

36
Q

What are the symptoms of maple urine

A

Muscle fatigue

Vomiting

Hyperactivity

Coma/death

37
Q

Why is muscle fatigue present in maple urine disease

A

Muscles prefer to use BCAA

38
Q

What causes Classic PKU

What does this convert normally

A

Phe hydroxylase deficiency

Autosomal recessive

F—>Y

39
Q

In classic PKU Whats used instead due to lack of PH

What does this produce as a biproduct

A

Tyrosine aminotransferase

Phenylpyruvate

40
Q

What can phenylpyruvate inhibit?

What does this lead to

A

Inhibits pyruvate decarboxylase in brain

Causes defect in myelin formation (retardation)

Because tyrosine is used to synthesise these products

41
Q

What is the treatment for Classic PKU

What are the symptoms

A

Limiting phenylalanine

Mousy odour

Fair skin (no tyrosine=no melanin)

42
Q

Which is the worst enzyme to have a defect in in the urea cycle

A

First enzymes

Affects all enzymes

43
Q

Tyrosinemia 1 leads to a build up of what

What does this interfere with

How do we treat it

A

Succinyl-acetoacetate

Haem synthesis

Nitisinone

44
Q

tyrosinemia 2

A

Tyrosine aminotransferase enzyme

Autosomal recessive

Corneal clouding / retardation

45
Q

Tyrosinemia 3 lacks what

A

HPD

46
Q

Alkaptonuria

What are the symptoms

How do we treat it

A

Homogenetisic Acid oxidase deficiency

Black pigmentation
Black urine

Vit.C reduces benzoquinone acetic Acid excretion
Nitisisone

47
Q

What happens to ketone bodies

A

Excreted our of urine/breath

Forms Acetyl CoA —>TCA

48
Q

Which two AA contain side chain sulphur

A

Methionine / cysteine

49
Q

What type of reaction forms tyrosine from phenylalanine

A

Condensation reaction

50
Q

What’s tyrosine used for

A

Neurotransmitters

Epinephrine

Dopamine

51
Q

Ornithine can produce what

A

Citrulline

Urea cycle

52
Q

What is glutathione produced from

A

Glutamate

Cysteine

glycine

53
Q

What does the production of glutathione need

A

Synthetases

54
Q

What type of bonds present in glutathione

A

Disulphide bridges

55
Q

What can tryptophan produce (3)

A

Nicotinate (NAD)

Indoeacetate (plant growth)

Serotonin (melatonin)

56
Q

Histidine can form what

What does it produce

What does it use

A

Histamine

CO2

Histidine decarboxylase +PLP

57
Q

Tyrosine —> dopamine —>

A

Epinephrine

58
Q

Dopa —>dopamine +CO2

A

Aromatic amino acid decarboxylase

59
Q

5-hydroxy tryptophan —>serotonin

A

Serotonin + CO2

Aromatic amino acid decarboxylase

Proteins (13 decks)

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