Biochemistry Flashcards

1
Q

what happens to unused amino acids and why?

A

they are degraded as there is no storage form for amino acids

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2
Q

what is the main site for amino acid degradation?

A

the liver

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3
Q

how does absorption of amino acids occur?

A

broken down from proteins by proteolytic enzymes in stomach and intestine

absorbed into intestinal cells and released into blood for absorption by other tissues

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4
Q

what regulates protein turnover?

A

AMP kinase

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5
Q

what does breakdown of amino acids produce and why?

A

ammonia (NH3) and ammonium ions (NH4+)

amino acids contain nitrogen

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6
Q

name three molecules that ammonia/ammonium are excreted in

A

urea
uric acid
creatinine

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7
Q

where is urea formed?

A

in the liver

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8
Q

what are the three steps in the synthesis of urea?

A

transamination
de-amination
urea cycle

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9
Q

what is transamination?

A

transfer of an amino from an AA to a ketoglutarate to make glutamic acid

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10
Q

what is de-amination?

A

removal of an amine from glutamic acid to make ammonia

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11
Q

what is the urea cycle?

A

ammonia is combined with aspartic acid to make urea

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12
Q

what are two major carriers of nitrogen in the blood to the liver?

A

alanine

glutamine

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13
Q

what happens to the carbon skeletons of amino acids?

A

they can be converted to glucose or oxidised in the TCA cycle

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14
Q

what do the carbon skeletons of ketogenic amino acids give rise to?

A

ketone bodies

fatty acids

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15
Q

name three inherited disorders of amino acid degradation

A

alkaptonuria
maple syrup urine disease
phenylketonuria

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16
Q

what is alcaptonuria?

A

the degradation of phenylalanine and tyrosine is blocked

17
Q

what happens in maple syrup urine disease?

A

degradation of valine, isoleucine and leucine is blocked, causing maple syrup smelling urine and mental and physical retardation

18
Q

how can maple syrup disease be prevented?

A

with an appropriate diet

19
Q

what happens in phenylketonuria?

A

phenylalanine accumulates in all body fluids which can lead to severe mental retardation

20
Q

how is PKU treated?

A

low phenylalanine diet