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Biochemistry > Kaplan Glycogen,gluconeovenesis And The Hexose Monophospahte Shunt > Flashcards

Kaplan Glycogen,gluconeovenesis And The Hexose Monophospahte Shunt Flashcards

1
Q

What happens to the glucose in the white(fast) muscles fibers and the red (slow)muscles fiber

A

In white muscles it is primarily converted to lactate

In red muscles it is all oxidized

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2
Q

What are the steps of glycogen synthesis

A

1- the G6P accumulate
2- the G6P is converted to G1P
3-G1P covered to UDP-glycose
3- which is converted to glycogen

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3
Q

What is the enzyme that convert G1P to UDP-glucose and what is co enzyme
And what happens

A

Co enzyme is UTP it takes out the extra P so the glucose could leave the the cells
and the co enzyme is UDP - glucose

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4
Q

What is called the enzyme the convert UDP-glucose to glycogen and what is co enzyme

A

It is called glycogen synthesis

Branching enzyme

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5
Q

Which hormone stimuli glycogen synthesis

A

Inslulin

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6
Q

What is are th steps of glycogenolysis

A

1-converting glycogen to G1P
2- G1P to G6P
3-G6P TO glucose

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7
Q

What is the name of the enzyme that convert glycogen to G1P and his coenzyme

A

It ia called glycogen phosphorylase

Co enzyme debranching

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8
Q

What is the name of the enzyme that convert G6P to glucose

A

Glucose 6 phosphate phosphatase

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9
Q

What are the stimulaters in that stimuli glycogen phosphorylase

A

Glucagon and epi and AMP

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10
Q

Where is the glucose 6 phosphote phosphatase enzyme exists

A

In the liver

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11
Q

What are the activaters of glycogen synthesis in liver and muscles

A

Liver insulin

Muscles insulin

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12
Q

What are the hormonal inhibitors of glycogen synthesis in liver and muscles

A

Liver glucagon and epi

Muscles epi

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13
Q

What are the activaters of glycogenolysis in liver and muscles

A

Liver glucagon and epi
Muscles epi and AMP
Ca via calmodulin

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14
Q

What is von gierke disease

A

It is G6P phosphatase deficiency

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15
Q

What are the characteristics of G6P phosphatase deficiency

A 
1- sever hypoglycemia 
2-hepatomegaly
3-lactic acidosis 
4-hyperlipidemia 
5-hyperuricemia 
6-fatty liver
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16
Q

What cause the hepatomegaly in the von gierke disease

A

Because the G6P is an osmotic product that will make the water get in liver cells

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17
Q

What cause the hyperlioidemia in the von gierke disease

A

Because the hypoglycemia the body will increase the levels if the epi and the glucagon which will degenerate the adipose tissues and release fatty acids into the blood

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18
Q

What cause the hyperuricemia in the von gierke disease

A

The AMP well be degenerate to uric acid the the lactic acid will slow down its release with the urin

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19
Q

Why does the fructose and the galactose ingestion will not increase the glucose levels in the blood

A

Because there final products will be G6P IN the end

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20
Q

What is the pompe disease

A

It is lysosomal alpha 1,4 glucosidase

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21
Q

What is the most noticeable characteristic of the pompe disease

A

Cardiomegaly

And death before reaching 2 years old

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22
Q

What is cori disease

A

It is debranching enzyme deficiency

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23
Q

What are the characteristics of cori disease

A

Hepatomegaly and mild hypoglycemia

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24
Q

What is andersen disease

A

It is branching enzyme deficiency

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25
Q

What are the characteristics of andersen disease

A

Hypotonia
Cirrhosis
Death by 2 yeasr

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26
Q

What is McArdel disease

A

It is muscles glycogen phosphorylase

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27
Q

What are the characteristics of Mc Aedle disease

A

Myoglobiuria

And muscles weekness and cramps after exercise

28
Q

What is hers disease

A

It is liver glycogen phosphorylase deficiency

29
Q

What are th characteristics of his disease

A

Hepatomegaly

Mild hypoglycemia

30
Q

What are the amino acids that are only ketogenic

A

Lysine and leucine

31
Q

What do we need to covert pyruvate to OAA

A

ABC

ATP
biotin(pyruvate carboxylasa)B7
Co2

32
Q

What is that get the OAA out of the mitochondria

A

Malate shunttle

33
Q

What is the name of the enzyme that convert OAA to PEP

A

PEP carboxykinase

34
Q

What is the enzyme that convert F1,6BP to F1P

A

It is F1,6BP phosphatase

35
Q

What is the name of the enzyme that convert G6P to glucose

A

G6P dehydrogenase

36
Q

What does the PEP carboxykinase

A

It needs GTP and it released CO2

37
Q

What are the hormones that effect PEP carboxykinase enzyme

A

Cortisol and glucagon

38
Q

How does the cortisol and the glucagon effet the PEP carboxykinase

A

In the genetic level

39
Q

What is the enzyme that is counter to F1,6BP enzyme

A

It is PFK1

40
Q

What does inhibit and stimuli the F1,6BPase enzyme

A

It gets stimuli ATP and inhibited by F2,6BP

41
Q

Does the gluconeogenesis require energy or not and where

A

Yes it does

In pyruvate carboxylase
And in PEP carboxykinase

42
Q

What is the drug that is used in diabetes type 2 to inhibit the gluconeogensis

A

It is metformin

43
Q

What are the sources of pyruvate in gluconeogenesis

A

1-lactate to pyruvate
2-glycerol -DHAP to F1,6BP
3-alanine aa to pyruvate

44
Q

What is the obligate activator of the pyruvate carboxylase enzyme

A

It is Acetyle-CaA from beta oxidation

45
Q

What is the malate shuttle

A

After we make the oxaloacetrate OAA in the mitochondria it can’t leave it so we convert to malate and reconverting to OAA in the cytoplasm

46
Q

Why is the gluconeogenesis is having only the alanine as a amino acid pathway what about the other amino acids?

A

All of them have his own pathway that ends in the in one of the kreps cycle acids then into malate then in to malate shuttle

47
Q

What is the source of the energy in the gluconeogenesis for liver

A

It is the fatty acids

48
Q

Can the Acetyle -CoA of the beta oxidation to be converted to glucose

A

No it can’t it can only produce ketone bodies

49
Q

Why is the alcohol is cause hypoglycemia and slow down the work of the gluconeogenesis

A

Well first the alcoholic do not eat very well
And the alcohol use Almost ALL the NAD to its dehydrogenase and leave no NAD to
1-lactate to be covered to pyruvate
2-and glycerol 3P to DAHP
3-and malate to OAA

50
Q

How the alcohol make the liver fatty

A

We due to the hypoglycemia the body will release glucagon which well break the adipose to fatty acids

And in the liver due to using all the NAD the glycerol 3P is accumulated and it will bind with the fatty acid and form triglycerides

51
Q

Why is it lethal to drink alcohol after exercise

A

Because the alcohol will use all the NAD and it will prevent converting of lactate to pyruvate which mean acidosis

52
Q

What ia called the enzyme that make the G6P inter the hexose monophaphate sunt

A

It is called G6PDH

53
Q

What is the name that is important to convert the ribose 5P to the other products

A

Transketolase

54
Q

Which enzymes needs thiamine pyrophosphate as co factor

A

Transketolase
Pyruvate dehydrogenase
Alpha ketoglutrate DH
branched chain ketoacid DH

55
Q

What is the transketolase deficiency called

A

Wernicke–Korsakoff syndrome (WKS)

56
Q

What is called the thiamine deficiency and what are the enzymes that are effected

A

It is called bri bir and the enzymes are thiamine pyrophosphate requiring enzymes

57
Q

What is the impotence if the HMP

A

it gives NADHP and another source for ribose 5-P

58
Q

What is the only enzyme the require thiamine in the RBCs

A

It is transketolase

59
Q

What is the importance of the NADHP in the liver

A

Synthetic of fatty acids ,cholesterol,nucleotides

60
Q

What is the importance of the NADPH in the phagocytic cells

A

It convert supra oxidant oxygen to hydrogen peroxide

61
Q

What is the importance of the G6PDH in the erythrocytes

A

It provides the glutathione reductase with the ions that it is going to give them to the glutathione to compensation the ions that it lost in converting the hydrogen peroxide to H2O

62
Q

How we get the H2O2 in the RBCs and the body deal with it

A

Everyday 0.5-3% of the Hp turned into met Hp because the extra oxygen steal the ion from the ferric iron and turn it to met Hp and release supra oxidant oxygen that we turn it to H2O2 because we can deal with by giving it ions from glutathione to form water

63
Q

What happens if H2O2 accumulated in the body

A

Hemolytic anemia

And heinz body

64
Q

What is heinz bodies ? And how them look under the microscope?

A

They are covertly cross linked Hb because the H2O2 making bonds between them .
And they look like a black dots under the microscope

65
Q

What is favism

A

It happens due that the patient having a partial G6PDH deficiency that is juat barely enough for the ROS that the body producing but when a person take a oxidant stress substance like fave bean or drug or infection
It gives him more ROS than can he deal with which cause a sever hemolytic anemia in 24-48 hours and jaudica and hemoglobinuria

Biochemistry (14 decks)

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