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Biochem > 1404 Renal Biochem > Flashcards

1404 Renal Biochem Flashcards

1
Q

What is anabolism?

A

Making of large molecules,requires energy

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2
Q

What is catabolism?

A

Break down of large molecules,releases energy

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3
Q

What is metabolism

A

Anabolism and catabolism

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4
Q

What is de novo/ endogenous?

A

Can make in house,so not essential

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5
Q

What are the non essential amino acids?

A

All girls go crazy after getting taken prom shopping
Alanine
Asparagine
Glutamate
Glutamine
Cysteine
Asparate
Glycine
Tyrosine
Proline
Serine

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6
Q

Which amino acids are nonessential (produced by the body) but are still classified as essential

A

Arginine
Methionine
Phenylalanine

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7
Q

Why is Arginine like this?

A

Arginine is synthesized by mammalian tissue but the rate is not sufficient to meet the required amount during growth

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8
Q

Why is methionine like this?

A

Methionine is required in large amounts to produce cysteine

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9
Q

Why is phenylalanine like this

A

Phenylalanine is needed in larger amnts to form tyrosine

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10
Q

Where are amino acid precursors present?

A

Amino acid precursors are intermediates in glycolysis, TCA,Kreisler Cycle,Citric Acid Cycle and PPP

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11
Q

What is transamination?

A

Transamination is the transfer of NH2 group between amino acid and a keto acid via a transaminase
Eg. Pyridoxal Phosphate

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12
Q

What is amination?

A

Transfer of NH2 groups between AAs using ATP and synthesizing enzymes

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13
Q

Why is arginine only essential to infants

A

Arginine is only essential to infants because of higher growth requirements

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14
Q

How do adults synthesize arginine

A

Adults synthesize arginine from glutamate

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15
Q

What is glutamate used to produced

A

Glutamate is used to produce proline

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16
Q

What is glutamate used to produce

A

Glutamate is used to produce arginine and prolime

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17
Q

How is serine produced?

A

3-physphoglycerate is oxidized to a ketoacid intermediate
Glutamate donates an amine group to form serine precursor
Phosphate removed from serine precursor to produce serine

18
Q

How is glycine produced?

A

Glycine is produced by removing an R group from serine
Serine - R Group —> Glycine

19
Q

How is cysteine produced?

A

Cysteine is synthesized by bacteria without the need for methionine

20
Q

How is cysteine also produced?

A

Methionine converted to homocysteine
Homocysteine then converted to cysteine

21
Q

How is tyrosine formed?

A

Tyrosine is formed by hydroxylating phenylalanine.Phe Catabolism

Tyrosine important for production of enzymes,hormones,melanin,E,NE,dopa.

22
Q

What is Garrods Hypothesis

A

Errors of amino acids are genetic disorders
Formed by mutations in gene coding enzyme
Symptoms results from too little AA production,toxic accumulation of intermediates or formation of alternative metabolites.

23
Q

What are the inborn errors associated with non essential amino acids

A

Alkaptonuria- Tyrosine

24
Q

What inborn errors are associated with essential amino acids?

A

Phenylketouria
Histindinemia(Histidine)

25
Q

What enzyme is mutuated in PKU

A

The enzyme phenylalanine hydroxylase is mutated in PKU

26
Q

What happens to excess Phe?

A

Excess Phe is shafted to other pathways where it is converted to other metabolites

27
Q

What are the symptoms of PKU

A

Reduced synthesis of neurotransmitters
Sever neural degradation
Mental retardation
Albinism
Musty odor in breath urine and skin

28
Q

What is albinism caused by?

A

Albinism is caused by a tyrosine deficiency

29
Q

How is tyrosine formed

A

Tyrosine is formed from phenylalanine via the enzyme phenylalanine hydroxlase

Phenylalanine—>Tyrosine

30
Q

What are the treatments for PKU?

A

Tyrosine Supplements
Limited Restriction of Phe
Gene Theraphy
Screening- pregnant women

31
Q

What enzyme is mutated in the disease Alkaptonuria?

A

Homogentisate oxidase is mutated in alkaptonuria

32
Q

What is the role of homogentisate oxidase?

A

This enzyme is involved in the Phe/Tyr catabolic pathway

33
Q

What is the effect of this mutation of homogentisate oxidase in Alkaptonuria?

A

Phe/Tyr not properly broken down and metabolites accumulate to toxic levels
Homogentisate/homogentisic acid accumulates in blood and tissues causing damage to heart valves
Accumulation in joints cause osteoarthritis

34
Q

What is ochronosis?

A

Excess homogentisate is converted to benzoquinone acid which is similar to melanin,giving tissues a dark colour

35
Q

Is homogentisate excreted ?

A

Homogentisate and its oxidized form alkapton are excreted in urine
Urine becomes dark in color
Even though excreted, levels of homogentisate in the body remain high

36
Q

What are the treatments for alkaptonuria?

A

Nitisinone
Anti inflammatory
Physical therapy
Manage Phe and Tyr intake

37
Q

How does nitisinone treat alkaptonuria?

A

Nitsinone reduces the level of homogentisic acid in the body

38
Q

What causes hyperthyroidism

A

Hyperthyroidism is caused by an accumulation of tyrosine
Increased metabolism and HR

39
Q

What is the cause of hypothyroidism?

A

Hypothyroidism is caused by a tyrosine deficiency
Slow metabolism and weight gain

40
Q

`what is the importance of histidine and histamine?

A

Hisitidine and histamine is important for rodents and human infants

41
Q

What is the role of THF

A

THF is an important cofactor for proper functioning of FTCD enzyme
Folic acid deficiency can lead to Histidinemia
THF needed for purine and Pyrimidines synthesis

42
Q

What are the treatments for Histidinemia?

A

Genetic screening
Low Histidine diet
Enzyme replacement therapy

Biochem (2 decks)

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