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Biochemistry > Lipids > Flashcards

Lipids Flashcards

1
Q

In the activation of FA, what is the enzyme responsible and where does this occur?

A

Fatty acyl CoA synthetase; cytosol

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2
Q

What are the immediate substrate and product of lipogenesis?

A

Acetyl CoA; Palmitoyl CoA

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3
Q

What is the rate limiting step of lipogenesis?

A

Acetyl CoA + HCO3 + ATP -> Malonyl CoA ( Acetyl CoA carboxylase)

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4
Q

What are the important steps of Fatty Acid Synthesis?

A

1) transport of acetyl coa to cytosol; 2) production of malonyl CoA; 3)elongation to palmitoyl CoA

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5
Q

What shuttle is used for the transport of acetyl coa to cytosol?

A

Citrate shuttle

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6
Q

Enzyme involved in the production of Malonyl CoA from Acetyl CoA?

A

Acetyl CoA carboxylase

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7
Q

What is the coenzyme needed for the production of Malonyl CoA?

A

Biotin

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8
Q

What is the sequence of steps repeated in the elongation of Palmitoyl CoA?

A

Condensation, Reduction, Dehydration, Reduction

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9
Q

What is required as donor of reducing equivalents in the reduction reactions of elongation of Palmitoyl CoA?

A

NADPH

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10
Q

What are the sources of NADPH?

A

PPP, Malic enzyme, Isocitrate dehydrogenase

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11
Q

Where is further elongation and desaturation happen in Lipogenesis?

A

smooth endoplasmic reticulum

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12
Q

What is the structure of triglycerol?

A

3 FA esterified to a molecule of glycerol

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13
Q

What are the steps of TGA synthesis?

A

1) sequential addition of 2 fatty acyl CoA to glycerol 3 phosphate; 2) removal of phosphate; 3) addition of the third fatty acyl CoA

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14
Q

What are the sources of glycerol 3 phosphste in TGA synthesis?

A

1) DHAP from glycolysis; 2) Phosphorylation of free glycerol

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15
Q

What is the enzyme needed to get DHAP from glycolysis and on which organs can this be seen?

A

Glycerol 3 phosphate dehydrogenase; liver and muscle

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16
Q

What enzyme is needed for the phosphorylation of free glycerol and on what organ is this seen?

A

Glycerol kinase, liver

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17
Q

What is the enzyme needed for the mobilization of fats during fasting?

A

Hormone sensitive lipase

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18
Q

Mobilization of fats are activated by?

A

glucagon and epinephrine (phosphorylation)

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19
Q

Mobilization of fats inhibited by?

A

Insulin (Dephosphorylation)

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20
Q

Where does fatty acid activation occur?

A

cytoplasm

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21
Q

Where does beta oxidation occur?

A

Mitochondria

22
Q

What are the substrate and products of beta oxidation?

A

Palmitate; 8 acetyl coa, 7NADH, 7FADH2

23
Q

What is the rate limiting step of beta oxidation?

A

Translocation of fatty acyl coa from cytosol to mitochondria ( Carnitine-palmitoyl transferase)

24
Q

In the degradation of fatty acyl coa, what is the sequence of repeated steps?

A

Oxidation -> Hydration -> Oxidation -> Thiolysis

25
Q

What is the enzymes collectively called in the degradation of fattyl acyl coa?

A

Fatty Acid Oxidase

26
Q

In FA with an odd number of carbons, what are the sequential enzymes involved?

A

Propionyl CoA carboxylase (biotin) and Methylmalonyl CoA mutase (vitamin B12)

27
Q

Results in the deficiency in fat soluble vitamins and essential fatty acids, manifests as steatorrhea

A

Lipid Malabsorption

28
Q

What disease is presented with hypoglycemia due to impaired fatty acid oxidation and lipid accumulation with muscular weakness?

A

carnitine deficiency

29
Q

Where does deficiency in carnitine palmitoyl transferase 1 affects?

A

Liver

30
Q

Where does carnitine palmitoyl transferase II deficiency affects?

A

cardiac and skeletal muscle

31
Q

Most common inborn error of fatty acid oxidation?

A

MCAD deficiency

32
Q

What is the toxin seen in jamaican vomiting sickness?

A

hypoglycin

33
Q

What is deficient in Refsum Disease?

A

phytanol CoA hydroxylase

34
Q

Known as cerebrohepatorenal syndrome; inherited absences of peroxisomes

A

Zellweger syndrome

35
Q

what disease is the inability to transport VLCFAs across the peroxisomal membranes leading to accumulation in the brain, adrenals, testes

A

Adrenoleukodystrophy

36
Q

What are the substrate and products of Ketogenesis?

A

Acetyl CoA; Acetoacetate, beta hydroxybutyrate, acetone

37
Q

What is the rate limiting step of ketogenesis?

A

acetoacetyl coa + acetyl coa -> hmg coa (HMG CoA synthase)

38
Q

Process of ketone body oxidation in the mitochondria of extrahepatic tissues

A

Ketolysis

39
Q

How is cholesterol stored?

A

Cholesterol esters

40
Q

What is the rate limiting step in cholesterol synthesis?

A

HMG-CoA -> Mevalonate ( HMG-CoA reductase)

41
Q

Statins are competitive inhibitors of?

A

HMG CoA reductase

42
Q

What is the most common form of CAH?

A

21 alpha hydroxylase deficiency

43
Q

11 Beta1 Hydroxylase Deficiency blocks which step in cholesterol pathway?

A

11 deoxycorticosterone to corticosterone

44
Q

In 21 alpha hydroxylase deficiency, which step in cholesterol pathway is blocked?

A

Progesterone to 11 deoxycosterone AND 17 hydroxyprogesterone to 11 deoxycortisol

45
Q

Three possible sources of amino acid pool

A

degradation and turnover of body protein, dietary intake, synthesis of nonessential amino acids

46
Q

What is the first phase of amino acid catabolism?

A

Transamination and Oxidative Deamination

47
Q

What are the enzyme and coenzyme involved in the 1st phase of amino acid catabolism?

A

aminotransferase; pyridoxal phosphate (B6)

48
Q

What are the two mechanisms of transport of ammonia from peripheral tissues?

A

through glutamine (glutamine synthetase); through alanina (glutaminase)

49
Q

What is the rate limiting step of urea cycle?

A

NH3 + CO2 -> carbamoyl phosphate (carbamoyl phosphate synthetase 1)

50
Q

What is the allosteric inhibitor of CPS 1?

A

N-acetylglutamate

51
Q

How many molecules of ATP is needed for 1 mol of urea?

A

3 mol ATP

Biochemistry (4 decks)

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