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Nutritional Biochem 1 > Copper > Flashcards

Copper Flashcards

1
Q

What are the two valence states that copper is found in?

A

Cuprous (Cu1+) or Cupric (Cu2+)

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2
Q

The copper content of the human body ranges between ___ to ___mg?

A

50-150mg

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3
Q

Copper is best absorbed in which state?

A

Cuprous (Cu1+) state

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4
Q

Copper is absorbed through the brush border using 3 different transports. What are they?

A
  1. Copper transporter 1 (Ctr1)
  2. DMT1 which symports Cu1+ and H+ simultaneously
  3. Amino acid transports for cysteine and histidine
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5
Q

High intakes of calcium and iron compete with which transporter?

A

DMT1

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6
Q

Much like Iron, Copper ions can damage cells through what reaction?

A

The non-enzymatic Fenton Reaction

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7
Q

Due to the Fenton Reaction issue, what 1 of 3 things is Copper always found to be bound to?

A
  1. Glutathione
  2. Metallothionein
  3. 1 of 6 chaperone proteins
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8
Q

What are the 6 chaperone proteins for Copper?

A
  1. Cox17
  2. Cox11
  3. Sco1
  4. Sco2
  5. Atox1
  6. CCS
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9
Q

There are four chaperone proteins that are specifically for Chytochrome C oxidase synthesis… which proteins are these?

A
  1. Cox17
  2. Cox11
  3. Sco1
  4. Sco2
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10
Q

What chaperone protein is used for Cu1+ [Cuprous] transport? (hint: 1 protein)

A
  1. Atox1
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11
Q

What chaperone protein is used for SOD (superoxide dismutase) synthesis? (hint: 1 protein)

A
  1. CCS [copper chaperone for superoxide dismutase]

* Note: CCS may be clinically relevant for assessing Cu status*

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12
Q

What two things are responsible for transporting Copper in the portal blood to the liver? (hint: 2 proteins)

A
  1. Albumin

2. Transcuprein

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13
Q

What Ferroxidase enzyme is responsible for delivering Copper to extrahepatic tissues?

A

Ceruloplasmin (Ferroxidase 1)

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14
Q

How many binding sites does Ceruloplasmin contain for copper?

A

6

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15
Q

When binding to Ceruloplasmin, what form does Copper need to be in? (Hint: Cu1+, Cu2+ etc.)

A

Either Cu1+ or Cu2+ will bind!

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16
Q

What percentage of circulating Copper released into the blood, from the liver, does Ceruloplasmin constitute?

A

60-70%

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17
Q

How is this Copper (Ceruloplasmin) then delivered to extrahepatic tissues?

A

Ceruloplasmin receptors

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18
Q

What is the main storage site for Copper, and what is it bound to?

A

Liver and it is bound to Metallothionein

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19
Q

How many Copper atoms can Metallothionein store?

A

12 atoms

20
Q

Being able to store this many Copper atoms helps decrease what affect Copper can have on the body?

A

Reduce oxidative effects of Copper when it produces superoxide and hydroxyl radicals!

21
Q

T/F Copper participates as an enzyme cofactor but not an allosteric component of enzymes…

A

FALSE. Copper participates as an enzyme cofactor OR an allosteric component of enzymes.

22
Q

Where does Copper function as an intermediate? (hint: pathway?)

A

ETC [electron transport chain]

23
Q

What are the 9 different metalloenzymes that require Copper that we cover in class?

A
  1. Ceruloplasmin (Ferroxidase 1)
  2. Cytochrome C Oxidase (aka Complex IV)
  3. Superoxide Dismutase
  4. Hephaestin
  5. Dopamine Hydroxylase (Dopamine monooxygenase)
  6. Monoamine oxidase (Amine oxidase)
  7. Tyrosinase
  8. Peptidylglycine a-amidating monooxygenase
  9. Lysyl Oxidase
24
Q

What is the glycoprotein that transports copper but is also known as an oxidative enzyme and antioxidant?

A

Ceruloplasmin (Ferroxidase 1)

25
Q

Ceruloplasmin (Ferroxidase 1) is responsible for the oxidation of which two minerals?

A

Ferrous (Fe2+) Iron and Manganese (Mn2+)

26
Q

Ferroxidase 1 is required to get iron to bind to transferrin for transport to what tissue, and for what type of synthesis?

A

Transport to Bone Marrow for HEMOGLOBIN synthesis

27
Q

Cytochrome C oxidase (aka complex IV) is specifically in what step of ETC?

A

Terminal oxidative step

28
Q

Cytochrome C oxidase contains 3 copper atoms per molecule, where are they located?

A

2 are located in CuA

1 is located in CuB

29
Q

Where is superoxide dismutase (SOD) found? (hint: think cell location)

A
  1. Cytosol of cells

2. Extracellularly

30
Q

Besides Copper, what other mineral is linked to this enzyme? (SOD)

A

Zinc

31
Q

Where is Copper specifically located on the SOD enzyme?

A

The active site

32
Q

If you were to remove copper, and not zinc what would happen to the activity of SOD?

A

REDUCED SOD activity

33
Q

What is the purpose of the enzyme Hephaestin, and what does it do with the mineral it transports?

A

It is essential for oxidizing iron to its Fe3+ state so that dietary iron can be transported from the enterocyte to the portal blood
[Note: Cu deficiency can lead to Anemia in relation to this mechanism]

34
Q

Dopamine Hydroxylase is also known as what?

A

Dopamine Monooxygenase

35
Q

How many Copper atoms does Dopamine Hydroxylase require per molecule [of dopamine hydroxylase]?

A

8 Copper atoms

36
Q

Copper functions as an allosteric structural component of what enzyme? This enzyme can also be inhibited by what type of drugs?

A 
Monoamine oxidase (aka Amine oxidase)
Inhibited by antidepressant drugs [MAOI's]
37
Q

Tyrosinase produces what substance?

A

Melanin

38
Q

What will a deficiency in Tyrosinase lead to?

A

Albinism

39
Q

Amidation of peptide hormones resulting in the cleavage of carboxy terminal of glycine which in turn will leave behind a terminal amide is the result of what metalloenzyme? [hint: super long but could be shortened to PG-a-A-M]

A

Peptidylglycine a-amidating monooxygenase

40
Q

What will generate cross-links between connective tissue proteins such as elastin and collagen?

A

Allysine

41
Q

What enzyme takes a Lysine residue and forms a collagen chain?

A

Lysyl oxidase

42
Q

Knowing now how Lysyl oxidase can affect collagen synthesis, a deficiency in what can be integral in different connective tissue defects?

A

Copper deficiency

[by way of not acting upon lysyl oxidase]

43
Q

What else is Allysine required to form, as well as what property of connective tissue is it crucial for?

A

Desmosine cross-links in ELASTIN

Crucial for STRETCHINESS of this connective tissue

44
Q

What does the liver use to excrete copper? (hint: it’s a P-type ATPase)

A

ATP7B

45
Q

What disease is characterized by defective biliary copper excretion, and is caused by an inherited disorder involving mutations in the gene for ATP7B?

A

Wilson’s disease

46
Q

What is another name for ATP7B?

A

Wilson’s ATPase

47
Q

Wilson’s disease is a genetic disorder characterized by copper toxicity. When patients have Wilson’s disease, what other mineral is recommended for them to take to decrease their copper absorption?

A

Zinc supplements (>40mg/day)

Nutritional Biochem 1 (6 decks)

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