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Clinical Chemistry II - 4378 > Test 2: Protein Metabolism > Flashcards

Test 2: Protein Metabolism Flashcards

1
Q

How many AA are needed for gluconeogenesis?

A

18

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2
Q

What two proteins cannot be used for gluconeogenesis?

A

leucine and lysine***

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3
Q

If the liver is not functioning properly, ammonia will _______ and urea will _______.

A

increase, decrease

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4
Q

What is the rate limiting enzyme of the urea cycle?

A

carbamoyl phosphate synthetase 1 (EC 6, 3, 4, 16)***

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5
Q

About ____% of urea is filtered and the rest is reabsorbed at the distal tubule.

A

40-50

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6
Q

What is the principle of the biuret test?

A

copper binds to peptide bond to measure AA

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7
Q

What is the alpha carbon?

A

the carbon connected to the functional group

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8
Q

a protein is an amino acid chain that consists of more than ____ peptides.

A

100

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9
Q

What makes a peptide bond?

A

NH2 group linking the w/
COOH group of another,
forming a peptide bond.

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10
Q

What are the essential AA?

A

: Histidine (His), Isoleucine (Ile), Leucine (Leu), Lysine (Lys), Methionine (Met),
Phenylalanine (Phe), Threonine (Thr), Tryptophan (Trp), Valine (Val)

9 in total

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11
Q

pH at which overall net charge is neutral and the proteins stops moving

A

Isoelectric point (pI)

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12
Q

AA: equal # of opposing charge
Ionizable group at physiological pH of ____

A

7.4

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13
Q

Protein synthesis is used to make what types of proteins?

A
  • plasma proteins
  • Intracellular proteins
  • Structural proteins
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14
Q

Non Protein Nitrogen compound synthesis includes…

A

Purine, Pyrimidine, porphyrins, Creatine, Urea, Histidine, Thyroxine, Epi, Coenzyme NAD+

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15
Q

Amino acids are used for ____% of the total energy supply.

A

12-20

this energy substrate is not as good as fat or glucose

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16
Q

Pyruvate is an intermediate in the conversion of what amino acids to acetyl-CoA?

A

threonine, glycine, Serine, Cysteine, and Alanine (catabolic pathway)

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17
Q

The catabolic pathways of what amino acids are long and complex?

A

lysine, tryptophan, phenylalanine, tyrosine, and leucine

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18
Q

The catabolic pathways of what amino acids eventually get converted to alpha-ketoglutarate?

A

Arginine, proline, Histidine, Glutamine, and Glutamate

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19
Q

-Propionyl-CoA and L-methylmalonyl-CoA are intermediates in conversion of these amino acids to succinyl-CoA
-Methylmalonyl-CoA mutase is a vitamin B12 requiring enzyme

A

catabolic pathways of Methionine, Isoleucine, and valine

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20
Q

What are the metabolic disorders of amino acid metabolism? (most are genetic)

A

-Phenylketonuria
-Tyrosinemia
-Alkaptonuria
-Maple syrup urine disease (MSUD)
-Isovaleric acidemia
-Homocystinuria
-Cystinuria

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21
Q

Inborn error of “PHENYLALANINE” METABOLISM

A

Phenylketonuria (PKU)

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22
Q

what prevents the conversion to tyrosine with PKU?

A

Defective phenylalanine hydroxylase*

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23
Q

PKU causes the accumulation of…

A

phenylalanine/phenylpyruvic acid (phenylketone)

Can lead to severe metabolic acidosis & neurological problems (effects dopamine)
■ Can be controlled by monitoring diet

24
Q

How is anion gap calculated?

A

sodium - (chloride + bicarbonate)

25
Inherited disorder that leads to tyrosine accumulation.
Tyrosinemia
26
what causes Tyrosinemia?
Loss/defect in enzyme that catabolizes tyrosine in liver ---> build up of tyrosine
27
Which Tyrosinemia is the most severe? What causes it?
Type 1 decreased levels of fumarylacetoacetate hydrolase
28
What are the symptoms of type 1 Tyrosinemia?
failure to thrive, jaundice, cabbage-like odor, nose bleeds (weak capillaries)* liver & kidney failure, neuropathy & hepatic tumors
29
(rare) deficiency of tyrosine aminotransferase.
Type 2 Tyrosinemia
30
What are the symptoms of type 2 Tyrosinemia?
Excessive tearing, photophobia, eye pain, lesions and mental retardation
31
(very rare) Deficiency of 4-hydroxyphenylpyruvate dioxygenase
Type 3 Tyrosinemia
32
What are the symptoms of type 3 Tyrosinemia?
mild retardation, seizures and intermittent ataxia
33
What is known as “Black urine” or “black bone” disease?
Alkaptonuria
34
What causes Alkaptonuria?
Defect in homogentisate oxidase
35
What happens physiologically with Alkaptonuria?
-Increased homogentisic acid (HGA) - intermediate breakdown product -HGA deposits in connective tissue, cartilage, & skin and heart - darkening of skin, urine and arthritis
36
Rare genetic condition that causes urine to turn black when exposed to air, kidney and prostate calculi, and *heart problems*
Alkaptonuria
37
What causes dopamine to be effected with PKU?
Phenylalanine cannot be converted tyrosine to make L-DOPA ---> dopamine
38
What are the three branched chain amino acids (BCAAs)?***
valine isoleucine leucine
39
Disorder that involves a defect in α-ketoacid dehydrogenase.
Maple syrup urine disease
40
Maple syrup urine disease: individual cannot properly break down ...
the BCAAs (Val, Ile, Leu) -Leads to an accumulation of the BCAAs & their respective keto-acids
41
What are the symptoms of Maple syrup urine disease?
-Poor feeding, vomiting, lethargy, seizures, and developmental delays. -Urine has a characteristic “maple syrup” odor to it.
42
Rare disorder involved in the proper catabolism of leucine
Isovaleric acidemia
43
Isovaleric acidemia leads to increased _____________.
isovaleric acid
44
Isovaleric acidemia: Defect in what enzyme?
isovaleryl-CoA dehydrogenase (IVD) enzyme
45
What are the symptoms of Isovaleric acidemia?
Poor feeding, sweaty feet, vomiting, lethargy, and progress to coma
46
What causes the distinctive odor of sweaty feet in Isovaleric acidemia?
Increase in isovaleric acid
47
Inherited disorder involving cystathionine β-synthase (CBS) **accumulation of homocysteine & methionine
Homocystinuria
48
What are the two cysteine disorders?
-Homocystinuria -Cystinuria
49
Individual cannot properly metabolize methionine/homocysteine
Homocystinuria
50
What are the symptoms of Homocystinuria?
lens dislocation in eye, increase in blood clots, skeletal abnormalities, & learning problems.
51
Condition characterized by build up of cysteine caculi in kidney & bladder ■ as a result of inability to reabsorb cysteine
Cystinuria
52
Is Cystinuria caused by a defective metabolic pathway?
NO
53
Cystinuria: Inherited disorder of cysteine transport ___________ (SLC3A1 or SLC7A9) ■ Defect in the transport of cysteine back in to the body
proteins (not enzymes)
54
What is an enzyme that does the same action as Methionine synthase?
Homocysteine Methyl transferase (Homocysteine ----> Methionine)
55
Conversion of homocysteine to cystathionine is done by....
Cystathionine Β-synthase (TRANSSULFULATION)
56
-Glutathione mobility to cells to replenish glutathione -important enzyme of the liver
Gamma glutamyl transferase (GGT)
57
What can an increased GGT level signify?
alcohol toxicity

Clinical Chemistry II - 4378 (28 decks)

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