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biochemistry > Unit 2 - Redone > Flashcards

Unit 2 - Redone Flashcards

1
Q

Humans contain combinations of how many amino acids for protein?

A

20 amino acids

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2
Q

How many amino acids are essential and cannot be synthesized?

A

9

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3
Q

What organs play a role in amino acid synthesis? What are the roles?

A

Liver and Kidneys
Transamination and deamination

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4
Q

Ampholyte

A

Ionized molecule with both negative and positive charges

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5
Q

Peptide bond

A

Bond between amino end of one a.a. and carboxyl end of next a.a.

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6
Q

Isoelectric point

A

pH where amino acid has no net charge

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7
Q

What determines an amino acid’s isoelectric point or pI?

A

R group

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8
Q

When a peptide bond forms, what releases?

A

Water

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9
Q

Which part of an amino acid is capable of donating hydrogen?

A

Amino group (NH3+)

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10
Q

Which part of amino acid is capable of accepting hydrogen?

A

Carboxyl group (COO-)

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11
Q

Why is water released from a peptide bond?

A

COOH gives up OH
NH2 gives up H

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12
Q

Oligopeptide

A

Short chain, up to 5 a.a.

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13
Q

Polypeptide

A

Chain of 6-30 a.a.

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14
Q

Proteins

A

Complex compounds with >40 a.a.

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15
Q

Proteome

A

Total array of proteins expressed by an organism’s genetic material under certain conditions

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16
Q

Aminoacidurias are either

A

Overflow or renal issues

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17
Q

Overflow

A

Blood/Plasma levels high
Renal threshold surpassed

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18
Q

Renal aminoacidurias

A

Blood/Plasma levels normal
Kidneys can’t reabsorb amino acid properly

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19
Q

Aminoacidopathies

A

Defect in handling parent amino acid (earlier in the line)

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20
Q

Organic acidemias

A

Defect in catabolic pathway (later down the line)

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21
Q

Normal phenylalanine handling

A

Phenylalanine + OH = Tyrosine

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22
Q

What is the main problem in PKU?

A

Virtually absent hydroxylase, can’t produce tyrosine

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23
Q

What is the product of PKU?

A

Instead of tyrosine, it’s toxic phenylpyruvate

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24
Q

What does toxic phenylpyruvate cause?

A

Failure to thrive in infants, can lead to irreversible retardation

25
Q

What is the fix for PKU?

A

Avoid milk or aspartame
Take tyrosine

26
Q

How is PKU identified?

A

Screening after sufficient milk feedings
OR
Mousy odor, blue-green color after addition of ferric chloride

27
Q

When is PKU usually diagnosed?

A

Early on, at birth

28
Q

What kind of amino acid issue is PKU?

A

Overflow, phenylalanine elevates in blood, CSF, urine

29
Q

Tyrosine is a precursor for

A

Thyroid hormones
Adrenal hormones
Melanine

30
Q

Tyrosinemia 1

A

Low fumarylactoacetate hydrolase
Near END of pathway

31
Q

Tyrosinemia 2

A

Low tyrosine aminotransferase
Near START of pathway

32
Q

Tyrosinemia 1 can cause

A

Failure to thrive
Liver/Spleen enlargement
Photophobia
Rickets
Liver disease/Cirrhosis
Tyrosine crystals in urine

33
Q

Tyrosinemia 2 can cause

A

Issues with eyes, skin, brain

34
Q

Alkaptonuria ususally diagnosed by

A

Urine blacken upon standing

35
Q

Alkaptonuria occurs because of

A

Homogentisic acid low or missing

36
Q

Why does urine darken in alkaptonuria

A

Alkaline urine, time/exposure to air

37
Q

Diagnosis of alkaptonuria

A

HGA levels in blood
Quick confirmation from adding base to fresh urine to see if it will darken

38
Q

Why does melanuria cause urine to blacken upon standing

A

Excess melanin from malignant melanoma

39
Q

What does tumor secrete in melanuria

A

5,6 dihyroxyindole

40
Q

Two major types of branched chain amino acid disorders

A

Maple syrup urine disease
organic acidemias

41
Q

Burnt sugar odor

A

Maple syrup urine disease

42
Q

Sweaty feet odor

A

Organic acidemias

43
Q

What kind of amino acid issue is cystinuria

A

Renal aminoaciduria

44
Q

What is the main issue in cystinuria

A

Cystine not reabsorbed by the kidney because of lack of enzyme

45
Q

Diagnosis of cystinuria

A

Increased cystine, lysine, arginine, ornithine
Cystine crystals in urine

46
Q

Cystinuria treatment

A

Keep urine at a n alkaline pH so crystals dont form

47
Q

Cystinosis

A

Accumulation of cystine in the tissues

48
Q

Two types of aminoacid urias

A

Overflow
Renal aminoaciduria

49
Q

Two types of inborn errors of metabolism of amino acids

A

Aminoacidopathy
Organic acidemia

50
Q

What is in excess in an aminoacidopathy?

A

Parent amino acid

51
Q

What is in excess in an organic acidemia?

A

Metabolic products

52
Q

Historic tests for IDing amino acid problems

A

Guthrie
Chemical tests
Urinalysis
Thin Layer Chromatography

53
Q

Guthrie test

A

Agar, everything but amino acid of interest
Bacillus subtitles
If organism grows, that means there is high amounts of that amino acid present

54
Q

Chemical testing

A

Color produced when reacts with ferric chloride

55
Q

TLC

A

Specimen on medium
Medium in solvent
Amino acids move based on interaction with solvent
a.a. ID’d based on color and position

56
Q

HPLC

A

As amino acids elute, they’re separated based on mass to charge ratio

57
Q

When is newborn screening done?

A

When it can be caught in 24-48 hours
Can’t be tested based on clinical symptoms
There’s a test to identify it leading to treatment and intervention

58
Q

Specimen requirements for newborn screening

A

Normal diet at least 24 hours prior
Fasting 6-8 hours

biochemistry (26 decks)

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