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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
How can we treat type 2 hyperammonaemia
High carbs / low protein(low AA+NH4) Benzoic Acid for ammonia detoxification
26
Arginosuccinate synthase deficiency What causes it Treatment
Citrullinemia Autosomal recessive Arginine enhances citrulline excretion
27
Arginosuccinic aciduria is caused by what? How do we treat it
Arginosuccinase defiency (Arginosuccinate —> arginine +fumerate) Arginine promotes nitrogen excretion
28
What causes arginosuccinic aciduria | What does it lead to for symptoms
Arginosuccinase deficiency Vomiting
29
Hyperargininaemia How do we treat it
Arginase deficiency Increased ammonia Retardation Rare autosomal recessive Low protein Low arginine
30
How do we treat hyperargininaemia
Low protein diet Restrict arginine intake
31
What can be used as treatments for urea disorders (2)
Benzoate Phenylacetate
32
How do benzoate / phenylacetate work
Converted into CoA thioesters Combine with glutamine +glycine Bind to ammonia Excreted in urine
33
Maple syrup urine disease can’t break down what 3 AA
Valine Isoleucine Leucine Chained AA
34
What are maple syrup urine patients lacking What causes it
Lacking BCK dehydrogenase Autosomal recessive 1 in 150,000
35
What causes the smell of maple syrup
Sotolone
36
What are the symptoms of maple urine
Muscle fatigue Vomiting Hyperactivity Coma/death
37
Why is muscle fatigue present in maple urine disease
Muscles prefer to use BCAA
38
What causes Classic PKU What does this convert normally
Phe hydroxylase deficiency Autosomal recessive F—>Y
39
In classic PKU Whats used instead due to lack of PH What does this produce as a biproduct
Tyrosine aminotransferase Phenylpyruvate
40
What can phenylpyruvate inhibit? What does this lead to
Inhibits pyruvate decarboxylase in brain Causes defect in myelin formation (retardation) Because tyrosine is used to synthesise these products
41
What is the treatment for Classic PKU What are the symptoms
Limiting phenylalanine Mousy odour Fair skin (no tyrosine=no melanin)
42
Which is the worst enzyme to have a defect in in the urea cycle
First enzymes Affects all enzymes
43
Tyrosinemia 1 leads to a build up of what What does this interfere with How do we treat it
Succinyl-acetoacetate Haem synthesis Nitisinone
44
tyrosinemia 2
Tyrosine aminotransferase enzyme Autosomal recessive Corneal clouding / retardation
45
Tyrosinemia 3 lacks what
HPD
46
Alkaptonuria What are the symptoms How do we treat it
Homogenetisic Acid oxidase deficiency Black pigmentation Black urine Vit.C reduces benzoquinone acetic Acid excretion Nitisisone
47
What happens to ketone bodies
Excreted our of urine/breath Forms Acetyl CoA —>TCA
48
Which two AA contain side chain sulphur
Methionine / cysteine
49
What type of reaction forms tyrosine from phenylalanine
Condensation reaction
50
What’s tyrosine used for
Neurotransmitters Epinephrine Dopamine
51
Ornithine can produce what
Citrulline Urea cycle
52
What is glutathione produced from
Glutamate Cysteine glycine
53
What does the production of glutathione need
Synthetases
54
What type of bonds present in glutathione
Disulphide bridges
55
What can tryptophan produce (3)
Nicotinate (NAD) Indoeacetate (plant growth) Serotonin (melatonin)
56
Histidine can form what What does it produce What does it use
Histamine CO2 Histidine decarboxylase +PLP
57
Tyrosine —> dopamine —>
Epinephrine
58
Dopa —>dopamine +CO2
Aromatic amino acid decarboxylase
59
5-hydroxy tryptophan —>serotonin
Serotonin + CO2 Aromatic amino acid decarboxylase

Proteins (13 decks)

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