Nitrogen Flashcards

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1
Q

What do amino acids and nucleotides contain that carbohydrates and fats don’t?

A

Nitrogen!

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2
Q

What is nitrogenase inactivated by?

A

Oxygen

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3
Q

Which is the only amino acid that can obtain its nitrogen directly from NH4?

A

Glutamate

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4
Q

What is the name of the process of incorporating nitrogen from glutamate into proteins and nucleotides?

A

Assimilation

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5
Q

How do organisms conserve N2?

A

Transferring amino groups between different molecules - transamination

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6
Q

Is transamination readily reversible?

A

Yes

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7
Q

Do transaminases participate in amino acid synthesis or degradation?

A

Both

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8
Q

What is pyridoxal phosphate?

A

A cofactor made from vitamin B6, required by all aminotransferases

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9
Q

Which enzymes transfer NH4 from glutamate to other amino acids?

A

Aminotransferases

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10
Q

Ammonia is transported in the bloodstream safely as _________

A

Glutamine

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11
Q

Why is glutamate made, then converted into glutamine or alanine, only to be converted back in the liver?

A

Glutamate is negatively charged, whereas alanine and glutamine are uncharged. Charged molecules don’t pass through membranes easily, hence conversion to uncharged molecules allows easy transport.

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12
Q

Where is excess glutamate metabolised?

A

Mitochondria of hepatocytes

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13
Q

What are the 9 essential amino acids needed in the diet?

A
Histidine 
Isoleucine 
Leucine
Lysine
Methionine
Phenylalanine
Threonine
Tryptophan
Valine
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14
Q

Which amino acids are both glucogenic and ketogenic?

A

Isoleucine, tyrosine and phenylalanine

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15
Q

Name four clinical features of inherited metabolic disorders presenting in childhood

A
  • Acidosis
  • Failure to thrive
  • Vomiting, refusal of feeds, irritability
  • Central nervous system dysfunction
  • Hypoglycaemia
  • Unusual odour
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16
Q

Which enzyme is missing in phenylketonuria?

A

Phenylalanine hydroxylase (PAH)

17
Q

Do all inherited metabolic disorders present clinically in infancy/childhood?

A

No - some may present in older patients

18
Q

True or false: All inherited metabolic disorders are untreatable.

A

False, many are treatable