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Biochem Fall > Chapter 16- Imi Biochem > Flashcards

Chapter 16- Imi Biochem Flashcards

1
Q

function of lipids

A

Thermal insulation Energy storage (as TG) Metabolic fuels Membrane components (Phospholipids & cholesterol) Hormones (steroids & vitamin D) Precursors of prostanoids & Leukotriens Vitamin A, D, E, & K Emulsifying agents in the digestion & absorption of lipids (bile acids) Surfactants in the alveolar membrane (phosphatidylcholine)

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2
Q

Non-polar lipids

A

triacylglycerols, cholesteryl esters

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3
Q

Polar lipids

A

Fatty acids, cholesterol, glycerophosphatides, glycosphingolipids

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4
Q

lauric acid carbons

A

12, produces net 95 ATP

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5
Q

myristic acid carbons

A

14, produces net 112 ATP

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6
Q

palmitic acid carbons

A

16, produces net 129 ATP

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7
Q

stearic acid carbons

A

18, produces net 146 ATP

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8
Q

arachidic acid carbons

A

20, produces net 163 ATP

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9
Q

oleic acid

A

18 carbons 1 double bond, produces net 144 ATP

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10
Q

linoleic acid

A

18 carbons 2 double bonds

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11
Q

arachidonic acid

A

omega 6, 20 carbons and 4 double bonds, produces net 155 ATP

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12
Q

increased FA oxidation characteristic of

A

starvation and DM producing ketone body production in the liver

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13
Q

during starvation what cycle is inhibited

A

TCA

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14
Q

3 acyl-CoA synthases

A

acetyl-CoA synthase medium-chain acyl-CoA synthase acyl-CoA synthase

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15
Q

how is acyl-CoA is transported into the mitochondrial matrix

A

acyl-CoA-synthase -> carnitine palmitoyl transferase I -> carnitine acylcarnitine translocate -> carnitine palmitoyl transferase II

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16
Q

Inhibitor of carnitine palmitoyl transferase I

A

Malonyl-CoA, a precursor in the synthesis of FAs, is an allosteric inhibitor of CPTI in the liver

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17
Q

Malonyl-CoA prevents what

A

futile cycle of simultaneous FA oxidation & synthesis

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18
Q

carnitine is synthesized from

A

Lys & Met

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19
Q

inherited defects of carnitine metabolism results in

A

impaired utilization of long chain fatty acid metabolism, hypoketotic hypoglycemia

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20
Q

beta-oxidation occurs in

A

mitochondria

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21
Q

defects in FA oxidation

A

mitochondrial electron transfer flavoprotein and ETF-ubiquinone oxidoreductase, turns in beta-oxidation spiral, activation to CoA derivatives carnitine mediated translocation into mitochondria

22
Q

beta oxidation energetics palmitic acid

A

C16, 8 reactions x12 = 96, 7 FADH2 produced x2= 14, 7 NADH produced x3= 21, total ATP produced=131, net ATP 130 due to 1 ATP initial investment

23
Q

carnitine palmitoyl transferase 1 in inhibited by

A

malonyl-CoA

24
Q

acetyl-CoA carboxylase is inhibited by

A

glucagon

25
Q

acetyl-CoA carboxylase is stimulated by

A

insulin, acyl-CoA

26
Q

glucagon ultimately does what in fatty acid oxidation

A

stimulates fatty acid oxidation by inhibiting acetyl-CoA carboxylase that catalyzes malonyl-CoA formation

27
Q

insulin ultimately does what in fatty acid oxidation

A

inhibits fatty acid oxidation by stimulating acetyl-CoA carboxylase that catalyzes malonyl-CoA formation

28
Q

peroxisomal fatty acid oxidation occurs in

A

VLCFA, 20 carbons or longer

29
Q

preliminary peroxisomal FA oxidation

A

a preliminary β-oxidation in peroxisomes, shortened FA to octanyl-CoA and acetyl-CoA then transported to mitochondria for further oxidation

30
Q

how many ATP does peroxisomal oxidation yield

A

none, all energy produced appears as heat

31
Q

initial peroxisomal FA oxidation catalyzed by

A

flavoprotein dehydrogenase

32
Q

FADH2 produced by flavoprotein dehydrogenase in peroxisomal FA oxidation is

A

oxidized by O2 to H2O2 by cats

33
Q

zellweger’s

A

defective formation of peroxisomes or enzymes resulting in accumulation of VLCFA in liver & CNS causing severe neurological symptoms, death

34
Q

neonatal adrenoleukodystrophy childhood adrenoleukodystrophy

A

abnormality in peroxisomal ATP-binding cassette transport membrane protein (ABCD1) for VLCFA causing accumulation of VLCFA in brain causing severe neurological symptoms, death

35
Q

product of odd-chain number FA oxidation

A

propionyl-CoA

36
Q

sources of propionyl-CoA

A

isoleucine, valine, methionine, threonine catabolism, or cholesterol side chain oxidation

37
Q

propionyl-CoA can be converted to

A

succinyl-CoA -> oxidized or converted to glucose by OAA and pyruvate or delta-ALA (precursor for porphyrin biosynthesis)

38
Q

formation of succinyl-CoA from propionyl requires

A

3 mitochondrial enzymes and 2 vitamins (biotin and B12)

39
Q

alpha oxidation involves

A

oxidation of LCFA to 2-OH FA’s (catabolism of branched chain FA)

40
Q

oxidation of LCFA to 2-OH FA’s are constituents of

A

brain lipids

41
Q

Refsum’s disease

A

deficiency of phytanoyl-CoA hydroxylase, a peroxisome phytanic acid storage disease, defect of α-oxidation

42
Q

Phytanic acid derived from

A

animal fat and cow’s milk, from chlorophyll

43
Q

Refsum’s disease characterized by

A

accumulation of phytanic acid

44
Q

symptoms of Refsum’s

A

retinitis pigmentosa, failing night vision, peripheral neuropathy, cerebellar ataxia

45
Q

ω-Oxidation

A

Oxidation of the C atom most remote from the carboxyl group in a FA

46
Q

ω-Oxidation catalyzed by

A

monooxygenase that require NADPH, O2, & cyt P-450

47
Q

ω-Oxidation occurs in

A

liver microsomes & some bacteria

48
Q

ω-Oxidation produce

A

dicarboxylic acids that are further oxidized from either end

49
Q

oxidation of mon and poly unsaturated FA’s produced how many ATP per double bond

A

2

50
Q

linoleum acid

A

omega-6

51
Q

linolitic acid

A

omega-3

Biochem Fall (13 decks)

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