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Clinical Chemistry Lecture MIDTERMS > Amino Acids > Flashcards

Amino Acids Flashcards

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1
Q

Their R groups are mostly Hydrocarbons (CH)

A

Non-polar hydrophobic

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2
Q

Their R groups are Alcohol, Phenol, Thiol, Amide

A

Polar and charged

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3
Q

Their R group can’t be seen but supposedly, the oxygen has a NEGATIVE charge

A

POLAR ACIDIC

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4
Q

positive charge can be seen its side chains

A

POLAR BASIC

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5
Q

When degraded = produce either Acetoacetyl-CoA/Acetyl-CoA

A

Ketogenic amino acids

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6
Q

Generate precursors of glucose.

A

Glucogenic amino acids

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7
Q

A class of inherited errors of metabolism in which there is an ENZYME DEFECT that inhibits the body’s ability to metabolize certain amino acids

A

AMINOACIDOPATHIES

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8
Q

PKU is due to the absence of enzyme —

A

Phenylalanine hydroxylase (PAH)

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9
Q

phenylalanine hydroxylase catalyzes the conversion of - to -

A

Phenylalanine to tyrosine

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10
Q

Increase of Phenylalanine on the
BLOOD

A

Hyperphenylalaninemia

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11
Q

There is a presence of
Phenylalanine in the URINE

A

Phenylketonuria

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12
Q

Characterized by its musty/mousy odor

A

PKU

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13
Q

derived from the DEAMINATION of phenylalanine

A

PHENYLPYRUVATE

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14
Q

a biochemical process that involves the REMOVAL OF AN AMINO GROUP (-NH2) from an organic compound, particularly an amino acid.

A

DEAMINATION

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15
Q

a type of chemical reaction in which two or more molecules combine to form a new compound that has an extended system of delocalized electrons.

A

CONJUGATION

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16
Q

Guthrie Bacterial Inhibition Assay

  • Bacteria that is incorporated in the agar plate
A

Spores of BACILLUS SUBTILIS

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17
Q

A semiquantitative, bacterial
inhibition assay for phenylalanine based on the ability of phenylalanine to facilitate bacterial growth in a culture medium despite the presence of a growth inhibitor.

A

GUTHRIE BACTERIAL INHIBITION ASSAY

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18
Q

direct measurement of phenylalanine in DRIED BLOOD FILTER DISKS

A

MICROFLUOROMETRIC ASSAY

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19
Q

Microfluorometric Assay

  • tes requires pretreatment of the blood filter disk w
A

Trichloroacetic acid

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20
Q

The procedure is based on the FLUORESCENCE OF A COMPLEX formed by phenylalanine, ninhydrin, and copper in the presence of a dipeptide (L-leucyl-L-alanine)

A

MICROFLUOROMETRIC ASSAY

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21
Q

gold standard for detecting a variety of congenital diseases in newborns.

A

TANDEM MASS SPECTROMETRY

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22
Q

This form of tyrosinemia is caused by a mutation in the FAH gene, which codes for the enzyme fumarylacetoacetate hydrolase (FAH)

A

Type 1 tyrosinemia

23
Q

caused by a mutation in the TAT gene, which leads to a deficiency of the enzyme tyrosine aminotransferase (TAT)

A

Type II tyrosinemia

24
Q

a rare disorder

caused by a mutation in the HPD gene that results in deficiency of the enzyme 4-hydroxyphenylpyruvate dioxygenase (HPD)

A

Type II tyrosinemia

25
Q

toxic metabolite that forms when tyrosine cannot be metabolized through the appropriate enzymatic pathway.

A

Succinylacetone

26
Q

Treatment for tyrosinemia

A

Low protein diet + nitisinone

27
Q

Alkaptonuria —> deficient in enzyme -

A

Homogentisate oxidase

28
Q

1st symptom of alkaptonuria

A

Arthritis like degeneration

29
Q

DARK BLUE BLACK PIGMENTATION when HGA is deposited in the cartilage of the ears, nose, and tendons of the extremities

A

OCHRONOSIS

30
Q

Maple syrup disease is due to the absence of a complex of enzymes known as

A

A-keto acid decarboxylase

31
Q

Screening fr maple syrup disease utilizes an agar plate containing —- for the modified Guthrie test

A

4-azaleucine

32
Q

Autosomal recessive disorder; mutation of the IVD gene

A

Isovaleric acidemia

33
Q

Treatment for Isovaleric acidemia

A
  • protein restrictive diet
  • oral accumulation of glycine and carnitine
34
Q

Mutation n the CBS gene
(deficiency of the enzyme
cystathionine β- synthase)

A

Homocystinuria

35
Q

This deficiency results in elevated plasma and urine concentrations of METHIONIEN and of the precursor HOMOCYSTEINE.

A

HOMOCYSTINURIA

36
Q

Treatment of homocystinuria

A

Reduce conc. Of methionine and high doses of vit b6

37
Q

Cyanide-Nitroprusside Urine Spot Test produce what color

A

Red purple color

38
Q

Confirmation of homocysteine

A

Silver-nitroprusside Test

39
Q

In Silver-nitroprusside Test— Silver nitrate reduces
homocysteine to form -

A

Reddish color

40
Q

Result of a mutation in the ASS1 gene
○ Lack of the enzyme argininosuccinic acid synthetase (ASS)

A

Type I citrullinemia

41
Q

Caused by a mutation in the SLC25A13
gene whch codes for prod of citrin

A

Type II citrullinemia

42
Q

cells are prevented from making citrin, which inhibits the urea cycle

A

Type II citrullinemia

43
Q

THe result of a mutation in the ASL gene
LACk the enzyme argininosuccinic acid lyase (ASL)

A

Argininosuccinic aciduria

44
Q

Characterized by inadequate reabsorption of cystine during urine formation in the kidneys

A

Cystinuria

45
Q

A RED-PURPLE COLOR on reaction with sulfhydryl groups for diagnosis of cystinuria

A

Cyanide nitroprusside test

46
Q

Aromatic urine odor

A

Normal

47
Q

Foul, ammonia like urine odor

A

Bacterial decomposition
Urinary tract Infection

48
Q

Fruity sweet urine odor

A

Ketones (diabetes mellitus, starvation, vomiting)

49
Q

Maple syrup burnt sugar urine odor

A

Maple syrup urine disease

50
Q

Mousy urine odor

A

Phenylketonurria

51
Q

Rancid urine disorder

A

Tyrosinemia

52
Q

Sweaty feet urinen odor

A

Isovaleric acidemia

53
Q

Cabbage urine dor

A

Methionine malabsorption

54
Q

Bleach urine odor

A

Contamination

Clinical Chemistry Lecture MIDTERMS (5 decks)

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