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Metabolism > 5 protein metabolism and diagnosis > Flashcards

5 protein metabolism and diagnosis Flashcards

1
Q

what is transamination?

A

AA synthesis via exchange of functional groups b/w AA and ketoacid (α-ketoglutarate)

products are glutamate and pyruvate

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2
Q

what is oxidative deamination?

A

glutamate converted back to ketoglutarate by glutamate dehydrogenase

products are NADPH (for FA synth) and ammonia (NH3) > quickly reacts w/ H and becomes ammonium (NH4) enters urea cycle

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3
Q

If Learned This Huge List May Prove Truly Valuable

A

Isoleucine
leucine
threonine
histidine
lysine
methionine
phenylalanine
tryptophan
valine

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4
Q

phenylketonuria PKU

A

inherited

large amount of phenylketones from phenylalanine

phenylalanine oxidised to tyrosine b/c phenylalanine hydroxylase (enzyme) is deficient

metabolised by other pathways to become phenylpyruvate

detected in urine or blood concentration

treat w/ diet low in phenylalanine

could cause lack of brain development

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5
Q

homocystinuria HCU

A

rare inherited autosomal recessive

defect in methionine metabolism

deficiency of cystathionine Beta synthase (CBS) enzyme

B/c CBS deficient, homocysteine levels increase converting to methionine

diagnosed w/ high levels of homocysteine/methionine in plasma and urine

treat w/ low diet in methionine

could cause connective tissue, muscle, CNS/CVS disorders

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6
Q

what amino acids are strictly ketogenic?

A

lysine and leucine

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7
Q

most common defect of urea cycle?

A

Ornithine Transcarboxylase (OCT) deficiency

X linked

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8
Q

What causes defects in the urea cycle?

A

Autosomal recessive disorders

cause deficiency of one of enzymes or partial loss of function

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9
Q

refeeding syndrome?

A

when pt has been on low protein diet (malnourished)

enzymes of urea cycle are downregulated

refeed 5-10kcal/kg/day raise gradually till full needs met

risk factors - low BMI (<16), unintentional weight loss (15%), or >=10 days w/ no or small nutritional intake

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10
Q

Why would insulin inhibit gluconeogenesis

A

Too much glucose outside of cell therefore no more glucose is needed hence inhibition of gluconeogenesis

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11
Q

Why must ammonia be converted to urea

A

It is toxic

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12
Q

Why is removing NH2 from amino acids important

A

Carbon skeleton of amino acid can be used in oxidative metabolism

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13
Q

Why is folate and Vitamin B12 used as treatment for homocystinuria

A

folate and Vitamin B12 promote conversion of homocysteine to methionine (essential amino acid)

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14
Q

Why does glucagon stimulate gluconeogenesis

A

Low glucose outside of cell
Stimulate production of glucose from alternative sources

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15
Q

Why can’t the muscle convert Glucose 6 phosphate into glucose

A

Muscle lacks glucose 6 phosphatase

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16
Q

Why are tyrosine supplements given to PKU

A

Since phenylalanine hydroxylase not present then phenylalanine not converted to tyrosine

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17
Q

which specific amino acids needed for glutathione

A

glycine
cysteine

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18
Q

which specific amino acids needed for catecholamines

A

Tyrosine

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19
Q

Where is glycogen stored in the liver

A

Hepatocytes

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20
Q

Where is glycogen stored in muscle

A

Intra and inter myofibrils

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21
Q

Where is defect in homocystinuria

A

Cystathionine beta synthase (CBS)

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22
Q

Where does gluconeogenesis occur

A

Liver

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23
Q

Where does deamination occur

A

Liver and kidney

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24
Q

Where do carbon atoms for non essential amino acid synthesis come from(3)

A

Intermediates of glycolysis
Pentose phosphate pathway
Krebs cycle

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25
Q

When is a branch point created

A

Every 8-10 glucose residues

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26
Q

What type of genetic disorder is PKU

A

autosomal recessive

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27
Q

What type of condition is homocystinurias

A

Autosomal recessive

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28
Q

What tissues need a absolute source of glucose

A

Erthrocytes
Leukocytes
Kidney medulla
Lens and cornea of eye

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29
Q

What stimulates Hormone sensitive lipase and adipose TAG lipase

A

Glucagon and adrenaline
Phosphorylation

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30
Q

What pathways do low tyrosine affect (5)

A

Dopamine
Adrenaline
Noradrenaline
Thyroid hormone
Protein synthesis

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31
Q

What occurs in deamination

A

NH2 released as ammonia can converted to ammonium ion and converted to urea

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32
Q

What occurs during intestinal epithelial cell recombination

A

Fatty acids and glycerol combine to form Triacylglycerol

33
Q

What molecules are required throughout the whole process of fatty acid synthesis

A

ATP and NADPH

34
Q

What molecule combines with fatty acids to travel in the blood

A

Albumin

35
Q

What is used a primer for glycogen structure

A

Glycogenin

36
Q

What is tyrosine needed for

A

Thyroid hormone
Melanin

37
Q

What is tryptophan needed for(3)

A

Nicotinamide
Serotonin
Melatonin

38
Q

What is treatment for PKU

A

Low phenylalanine diet
Tyrosine supplements

39
Q

What is treatment for homocystinuria

A

Low methionine diet
Cysteine
Vitamins B6
Folate

40
Q

What is transamination

A

Move NH2 from amino acid to ketoacid to create a new amino acid

41
Q

What is the structure of glycogen

A

Polymer of glucose residues

42
Q

What is the process of fatty acid synthesis

A

Glucose to pyruvate in cytoplasm
Pyruvate to Acetyl-CoA and oxaloacetate in mitochondria
Oxaloacetate and acetyl-CoA to citrate
Citrate cleaved to Acetyl-CoA and oxaloacetate
Acetyl-CoA carboxylase produce malonyl-CoA from Acetyl-CoA
Fatty acid synthase complex build fatty acids through addition of 2 carbon units provided by malonyl-CoA

43
Q

What is the process of ammonia transport

A

Ammonia to glutamate
Glutamate to glutamine via glutamine synthetase
Glutamine goes from tissue to blood to liver
Glutamine to glutamate via glutaminase
Glutamate to ammonia to urea via glutamate dehydrogenase

44
Q

What is the name for fatty acid synthesis

A

Lipogenesis

45
Q

What is the enzyme required to convert glucose 6 phosphate to glucose 1 phosphate

A

Phosphoglucomutase

46
Q

What is the effect of insulin on gluconeogenesis enzymes

A

Decreased amount of PEPCK and Fructose 1,6 bisphosphatase

47
Q

What is the effect of glucagon on gluconeogenesis enzymes

A

Increased amount of PEPCK and Fructose 1-6-bisphosphatase
Stimulate gluconeogenesis

48
Q

What is some examples of ketogenic amino acids

A

Lysine
Leucine

49
Q

What is some example of amino acids that are both glucogenic and ketogenic

A

Tryptophan
Isoleucine
Threonine

50
Q

What is pyruvate converted to in fatty acid synthesis

A

Acetyl-CoA and oxaloacetate in mitochondria

51
Q

What is glutamate needed for

A

GABA

52
Q

What is glucose converted to in fatty acid synthesis

A

Pyruvate in cytoplasm

53
Q

What is glucose 6 phosphate converted to in the liver

A

Glucose

54
Q

What is an example of disorder caused by excess glycogen storage

A

Von gierke disorder

55
Q

What is an example of a condition that is caused by lack of glycogen degradation

A

McArdle disease

56
Q

What is acetyl-CoA and oxaloacetate condensed into in fatty acid synthesis

A

Citrate

57
Q

What hormones convert Triacylglycerol in adipose tissue to glycerol and fatty acids in blood (2)

A

Hormone sensitive lipase
Adipose TAG Lipase

58
Q

What happens to glucose 6 phosphate in the muscle

A

Enters glycolysis to produce lactate

59
Q

What happens to citrate in fatty acid synthesis

A

Enters cytoplasm and returns to acetyl-CoA and Oxaloacetate

60
Q

What goes wrong in phenylketonuria

A

Accumulation of phenylalanine
Phenylketones in urine
Phenylketones oxidised to phenylacetate

61
Q

What goes wrong in homocystinurias

A

High homocysteine and methionine
Homocysteine excreted in urine

62
Q

What enzymes are used in transamination

A

Aminotransferase

63
Q

What enzyme is used to convert glucose-6 phosphate to glucose

A

Glucose 6 phosphatase

64
Q

What enzyme is used to convert glucose to glucose 6 phosphate

A

Hexokinase in most cells
Glucokinase in liver

65
Q

What does homocystinuria affect(4)

A

Connective tissue
Muscle
CNS
CVS

66
Q

What does fatty acid synthase complex do

A

Produce fatty acids through addition of 2 carbon units from malonyl-CoA

67
Q

What does aspartate aminotransferase (AST) do

A

Convert aspartate to glutamate

68
Q

What does Alanine aminotransferase (ALT) do

A

Convert alanine to glutamate

69
Q

What does a low creatinine in blood suggest

A

Muscle wastage

70
Q

What does a high creatinine in blood suggest

A

Damage to nephrons

71
Q

What deficiency is present in PKU

A

Phenylalanine hydroxylase

72
Q

What can partial loss of enzyme function in urea cycle lead to

A

Hyperammonaemia
Accumulation of urea cycle intermediates

73
Q

What can free amino acids be converted to in the liver(2)

A

Amino group
Carbon skeleton

74
Q

What are the enzymes that can deaminate amino acids (3)

A

Amino acid oxidase
Glutaminase
Glutamate dehydrogenase

75
Q

what are some symptoms of ornithine transcarbamylase deficiency (OCT)

A

vomiting
lethargy
seizures
coma

76
Q

What 2 enzymes in gluconeogenesis are controlled by hormones

A

PEPCK
Fructose 1,6 bisphosphatase

77
Q

What 2 enzymes are rate limiting in glycogen metabolism

A

Glycogen synthase
Glycogen phosphorylase

78
Q

The enzyme Ornithine transcarbamylase leads that what disorder

A

Ornithine transcarbamylase deficiency

79
Q

Plasma levels of ALT and ASL are used to measure function of what organ

A

Liver

Metabolism (9 decks)

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