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Biochemistry > Test3- Diseases and cofactors > Flashcards

Test3- Diseases and cofactors Flashcards

1
Q

Carboxlyases use what as a cofactor?

A

Biotin

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2
Q

What does Methylmalonyl CoA mutase need as a cofactor

A

Deoxyadenosyl cobalamin (B12)

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3
Q

What is the rate limiting enzyme of lipolysis

A

Hormone sensitive lipase

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4
Q

Hormone sensitive lipase is ________ when it is on

A

Phosphorylated

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5
Q

What is the rate limiting enzyme of glycolysis?

A

PFK-1

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6
Q

What is hexokinase inhibited by

A

G6P (its product)

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7
Q

What is glucokinase inhibited by?

A

Nothing, no product inhibition

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8
Q

What is PNDM from?

A
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9
Q

What pathway is active when PFK-2/FBP-2 is phosphorylated?

A

Gluconeogenesis

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10
Q

PFK-2 is ________ when active

A

Dephosphorylated

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11
Q

Pyruvate kinase is active when it is ______

A

Dephosphorylated

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12
Q

PDHC is active when _______

A

Dephosphorylated

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13
Q

What happens with a PDHC deficiency?

A
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14
Q

What happens with a thiamine deficiency? What is the syndrome that can manifest?

A
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15
Q

What is the rate limiting enzyme of TCA cycle?

A

Isocitrate dehydrogenase

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16
Q

What happens with arsenic poisoning?

A
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17
Q

What is LHON?

A
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18
Q

What is Leigh syndrome? What is the presentation?

A
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19
Q

What are the inhibitors of the ETC and what are the important examples and treatment?

A
20
Q

What are the examples of uncouplers of the ETC and the consequences? What are the natural uncouplers?

A
21
Q

What happens with hyperthyroidism?

A
22
Q

What happens with atractyloside poisoning?

A
23
Q

What is the rate limiting enzyme of glycogenesis?

A

Glycogen synthase

24
Q

Where does glycogenesis occur

A

Cytosol

25
Q

Where does Liver Glycogenolysis occur

A

Cytosol

26
Q

What is the rate limiting enzyme of Liver Glycogenolysis

A

Glycogen phosphorlyase

27
Q

What is the cofactor needed by glycogen phosphorylase?

A

Pyridoxal phosphate B6

28
Q

Where does gluconeogenesis occur

A

In the liver and kidney cortex, in mitochondrial matrix and cytosol

29
Q

What is the rate limiting enzyme of gluconeogenesis?

A

FBPase-1

30
Q

What happens with a biotin deficiency?

A
31
Q

What happens with a PEPCK deficiency?

A
32
Q

What is the rate limiting enzyme of the HMP shunt?

A

G6PD

33
Q

Where does the HMP shunt take place?

A

Cytosolic pathway in all cells

34
Q

What happens with a GALT deficiency?

A
35
Q

What happens with an aldolase B deficiency?

A
36
Q

What happens with low insulin levels? What does Niacin inhibit?

A
37
Q

What is the rate limiting enzyme for Beta oxidation?

A

Carnitine shuttle (CPT-I
enzyme*)

38
Q

Where does Beta oxidation take place

A

Mitochondria

39
Q

What is the rate limiting enzyme of ketogenesis?

A

HMG-CoA synthase

40
Q

What happens with continuously low I/G ratio?

A
41
Q

What is Jamaican vomiting sickness?

A
42
Q

What is the rate limiting enzyme of Lipogenesis

A

Acetyl-CoA carboxylase

43
Q

Where does Lipogenesis occur

A

Liver, cytosol on surface of SER

44
Q

Decrease in bile salts can be from what

A

Cholestasis from liver or bile duct

45
Q

Decrease in pancreatic enzymes can be from what

A

Cystic fibrosis, pancreatitis, EPI

46
Q

Decreased lipid absorption can be from what

A

IBD

Biochemistry (21 decks)

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