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M2015/16 > Biochem p. 104-108 > Flashcards

Biochem p. 104-108 Flashcards

1
Q

AAs required during periods of

growth?

A

Arg and His “HAl is tall”

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2
Q

AAs that are in histones, which bind negatively charged DNA?

A

Arg and Lys

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3
Q

Excess —— is generated by urea cycle and

converted to urea and excreted by the kidneys.

A

nitrogen (NH3)

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4
Q

which enzyme is used to produce Carbamoyl

phosphate?

A 
Carbamoyl phosphate synthetase I (mitochindria) 
CPs II (cytoplasm)
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5
Q

What is the required co-factor of Carbamoyl phosphate synthetase I?

A

N-acetylglutamate “NAG”

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6
Q

where does urea cycle occur?

A

mitochondria, cytoplasm

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7
Q

Using what enzyme and which substrate does carbamoyl phosphate become Citruline?

A

Ornithine transcarbamylase

Ornathine + Carbamoyl phosphate

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8
Q

which AAs is added into the Urea cycle?

A

Aspartate

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9
Q

Transport of ammonia by alanine and glutamate happens in which 2 cell types?

A

Muscle

Liver

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10
Q

Absence of N-acetylglutamate cause what path?

A

hyperammonemia.

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11
Q

Neonates that has poorly regulated respiration and body temperature, poor feeding, developmental delay and intellectual disability is seen in which 2 pathologies?

A

Carbamoyl phosphate synthetase I deficiency and N-acetylglutamate synth def both cause Hyperammonia.

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12
Q

T or F? The most common urea cycle disorder is a autosomal recessive disorder.

A

F! OTC is X-linked recessive

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13
Q

3 Finding in Ornithine transcarbamylase deficiency?

A

Orotic acid in blood and urine
decr BUN
symptoms of hyperammonemia

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14
Q

Excess of —– is converted to orotic acid which is part of the —– synthesis pathway.

A

carbamoyl phosphate, pyrimidine

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15
Q

Hypothyroid pts might be deficient in which AA?

A

Phenylalanine –> tyroxine

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16
Q

Pts having trouble with sleep might be deficient in what AA?

A

Tryptophan –> melatonin

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17
Q

Pts with porphyrias might have what AA def.?

A

Glycine –> Heme

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18
Q

G6PD dz might occur due to what AA def?

A

Glutamate –> Glutathion

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19
Q

Def of what AA can dec BUN and creatine?

A

Argenine

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20
Q

Male pt with angina and ED might have what AA deficiency?

A

Argenine… no NO production in the vessles

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21
Q

Pt suffering from albinism might be def in what AA?

A

Phenylalanine –> melanine

22
Q

Pt with Parkinson disease might be def in which 2 AA?

A

Phenylalanine and Tyrosine –> dopamin

23
Q

Which AA is constantly converted to its 2nd end product in Locus ceruleus in a pt with anxiety?

A

Phenylalanine–> NE–>EPi

24
Q

In theory, if one is trying to increase his/her HDL levels, which AA and its cofactor are needed?

A

Tryptophan –> niacin using Vit B2 and vit B6

25
Q

Severe vit B6 deficiency can theoretically worsen which 2 neurological disorders?

A

anxiety
Huntington disease
decr GABA

26
Q

Which cofactors are needed to produce a neurotransmitter which is decr in anxiety and depression

A

Vit B6, BH4 used to make serotonin from tryptophan

27
Q

AA used to produce neurotransmitter produced in Nucleus accumbens?

A

Glutamate makes GABA

28
Q

Which AA is in excess and which is essential in PKU?

A

excess: phenylalanine
essential: tyrosine

29
Q

Def of tetrahydrobiopterin cofactor is see in which path?

A

malignant PKU

30
Q

Ž Musty body odor is usually seen in

A

Disorder of aromatic amino acid metabolism

31
Q

which artificial sweetener must PKU pts avoid?

A

aspartame, which contains phenylalanine.

32
Q

(4) Finding is maternal PKU?

A

microcephaly
intellectual disability
growth retardation
congenital heart defects

33
Q

Findings of dark connective tissue, brown pigmented sclerae, urine turns black on prolonged exposure to air are seen in a pathology that has an enzyme def in degradation which AA?

A

Tyrosine, in Alkaptonurea

34
Q

Homogentisic acid is toxic to what type of collagen that causes debilitating arthralgias?

A

type ii for Cartilage

35
Q

Enzyme def in Alkaptonurea?

A

Homogentisate oxidase

36
Q

Enzyme deficiency in Maple Syrup dz (MSD)?

A

 α-ketoacid dehydrogenase

37
Q

Degradation of which branched amino

acids are blocked MSD? which one the most?

A

Isoleucine, Leucine, Valine— Leucine most

38
Q

What should be supplemented in MSD?

A

thiamine supplementation (vit B1)

39
Q

Which dz has this finding: intellectual disability, osteoporosis, marfanoid habitus,
kyphosis, lens subluxation, thrombosis, and atherosclerosis (stroke and MI).

A

Homocystinuria

40
Q

What other dz do we find lens subluxation besides homocystinurea and how do we differentiate btw them?

A

Homocystinurea — downward and in

Marfan synd — upward and out

41
Q

Name the 2 different enzyme deficiencies that can cause homocystinurea?

A

Homocysteine methyltransferase

Cystathionine synthase

42
Q

What is another cause of homocystinurea?

A

decr affinity of cystathionine synthase for
pyridoxal phosphate/vit B6.
Tx:  B6 and cysteine in diet

43
Q

Which type of the homocystinureas can be treated with incr  B12 and folate in diet?

A

the one with Cystathionine synthase defi

44
Q

Cystinuria is a hereditary defect of renal — and intestinal
AA transporter that prevents reabsorption of:

A

PCT

Cysteine, Ornithine, Lysine, and Arginine (COLA).

45
Q

How is Cystinuria diagnosed?

A

via Urinary cyanide-nitroprusside test

46
Q

Tx of cystine stones?

A 
urinary alkalinization (e.g., potassium citrate, acetazolamide)
chelating agents (e.g., penicillamine)
47
Q

Pt with intellectual disability, growth

retardation, seizures, fair skin, eczema, musty body odor is missing either an enzyme or a cofactor? what are thos?

A

phenylalanine hydroxylase
or
tetrahydrobiopterin cofactor

48
Q

(7) findings in Homocystinurea:

A 
intellectual disability
osteoporosis
kyphosis
marfanoid habitus
lens subluxation
thrombosis
atherosclerosis (stroke and MI).
49
Q

Methionine synthase is seen in what dz? what another name for this enzyme?

A

Homocystinurea

homocysteine methyltransferase

50
Q

How can strict vegans get an MI or stroke?

A

Vit B12 deficiency can cause Homocysteine to built up and cause atherosclerosis => MI or stroke.

51
Q

Describe what Homocysteine converts into via which 2 enzymes:

A

Methionine Cystathionine –**–> Cysteine

  • methionine synthase, vit B12
    • cystathionine synthase, Vit 6, Serine

M2015/16 (27 decks)

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