Lipid Biochemistry II Flashcards

1
Q

muscle weakness in arms and legs, elevated triglycerides with primary long chain FAs, lipid vacuoles in muscles

A

carnitine deficiency

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2
Q

glucagon

A

does not stimulate lipid release

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3
Q

insulin

A

stimulate lipid release

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4
Q

increased hormone sensitive lipase

A

TGL > glycerol + FAs

decreased insulin stimulates, as well as increased epi and increased cortisol

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5
Q

fatty acids

A

adipose tissue

  • carried with albumin in blood
  • to liver

-beta oxidation to acetyl CoA**

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6
Q

acetyl CoA

A

can go to ketone bodies
-brain and cardiac muscle

or to citric acid cycle

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7
Q

alpha oxidation

A

peroxisomes

-phytanic acid breakdown

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8
Q

omega oxidation

A

in some species of animals

alternate path to beta

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9
Q

beta-oxidation

A

long chain FA breakdown

in mito to produce acetyl CoA

to TCA cycle - to ETC > ATP

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10
Q

short chain FAs

A

2-4C

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11
Q

medium chain FAs

A

6-12C

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12
Q

short and medium chain FAs

A

diffuse freely into mitochondria

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13
Q

long chain FAs

A

14-20C

-activated first then transported into mito by carnitine shuttle to be oxidized

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14
Q

very long chain FAs

A

> 20C

  • enter peroxisomes
  • unknown mechanisms
  • oxidation occurs
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15
Q

fatty acid beta oxidation pathway

A

first in cytosol

long chain FA - activated by ATp to fatty acyl CoA

  • forms AMP (2 energy bonds)
  • in cytosol

-cytosolic fatty acyl CoA reacts with carnitine - fatty acyl carnitine (CAT1 or CPT1)

passes across inner mitochondria membrane via carnitine acylcarnitine tranferase

CAT II, or CPT II, converts fatty acyl carnitine to fatty acyl CoA in matrix

to beta oxidation

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16
Q

CPT1

A

outer mito membrane
-fatty acyl CoA to fatty acylcarnitine

-then transported across inner mito membrane

aka CAT1

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17
Q

CPT II

A

inner mito membrane

fatty acylcarnitine converted back to fatty acyl CoA in mitochondrial matrix

aka CAT2

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18
Q

fatty acyl CoA synthetase

A

FA + CoA > fattyl acyl CoA

outer mito membrane

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19
Q

myopathic CAT/CPT deficiency

A

problem with long chain FA metabolism

muscle aches, weakness, myoglobinuria, provoked by exercise and fasting

elevated muscle triglyceride**

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20
Q

MCAD

A

medium chain acyl-CoA DH deficiency

fasting hypoglycemia
no ketone bodies
vomiting
coma, death

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21
Q

carnitine

A

can be measured in blood

if present in blood - either CPT1 or CPT2 deficiency

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22
Q

CPT1 and 2 deficiency treatment

A

avoid fasting
dietary restric long chain FAs
carnitine supplement

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23
Q

muscle weakness with exercise, hypoerammonemia, death

A

CPT II deficiency

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24
Q

fasting hypoglycemia, inability to use LCFAs as fuel by liver

A

CPT I defiency

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25
Q

medium chain FAs

A

do not require carnitine shuttle

26
Q

liver disorder

A

likely more involvement of carnitine deficiency than CPT1 or CPT2

27
Q

causes of carnitine deficiency

A
  • inadequate in diet
  • enzyme deficiency (CPT)
  • diarrhea, diuresis, hemodialysis
  • hereditary leakage from renal tubules (primary)
  • increased carnitine requirements - ketosis or demand high
  • decreasead muscle carnitine - mito impairment
28
Q

Tx of carnitine deficiency

A

pharm carnitine oral dose

avoid fasting/exercise

supp medium chain FA

high carb, low fat diet

29
Q

browin urine, pain in arms and leg muscle when exercise, recommended carnitine supplement - likely diagnosis?

A

CPT II deficiency

30
Q

CPT I deficiency

A

with liver dysfunction

31
Q

MCAD

A

usually first 3-5 years of life

32
Q

tall stature - long bones

A

marfan

33
Q

brown urine and muscle weakness

A

CPT II deficiency

34
Q

xanthomas

A

accumulation of lipid-laden macrophages

from altered lipid metabolism or local cell dysfunction

35
Q

hyperlipoproteinemia

A

cause of xanthomas

-primary and secondary

36
Q

primary hyperlipoproteinemia

A

5 phenotypes

-also decreased synthesis of HDL or hepatic lipase deficiency

37
Q

type I hyperlipidemia

A

familial lipoprotein lipase deficiency

  • accumulation of chylomicrons
  • early childhood
  • acute pancreatitis
  • eruptive xanthomas
  • elevated plasma triglycerides
38
Q

type III hyperlipidemia

A

familial dysbeta-lipoproteinemia

  • accumulation of IDL
  • both triglyceride and cholesterol increase
  • mutation of apo E

premature atherosclerosis and xanthomas

39
Q

type IV hyperlipidemia

A

familial hypertriglyceridemia

  • over-production of VLDL
  • elevated plasma triglyceride levels
  • cholesterol low

eruptive xanthomas
coronary heart disease
DM II, obesity, alcoholism

40
Q

type IIa hyperlipidemia

A

accumulation of LDL

  • LDL receptor deficiency
  • defective apo B100
  • elevated plasma cholesterol
  • normal plasma triglyceride
  • severe atherosclerosis

**tendinous xanthomas, tuberous xanthomas, xanthelasmas

41
Q

type IIb hyperlipidemia

A

accumulation of LDL and VLDL

  • defective apoB100
  • variable levels of triglyceries and cholesterol

tendinous xanthomas, tuberous xanthomas, xanthelasma

42
Q

apo B100

A

for storage in adipose tissue

43
Q

apo B48

A

chylomicron

dietary lipid

44
Q

apoproteins

A

synthesized in liver
-mixed with lacteals (chylomicrons) in heart

-with protein on outside

45
Q

type V hyperlipidemia

A

genetic defect in apo-lipoprotein CII

  • accumulation of chylomicrons and VLDL
  • elevated triglyceride
  • early childhood
  • acute pancreatitis
  • eruptive xanthomas
46
Q

decreased synthesis of HDL

A

decrased apo A1 and apo CII

decreased reversed cholesterol transport

increased LDL

premature CAD

plane xanthomas

47
Q

hepatic lipase deficiency

A

accumulation of large triacyl-glycerol rich HDL and VLDL

coronary heart disease
xanthomas

48
Q

cutaneous xanthomas

A

with hyperlipidemia

49
Q

xanthelesma palpebrarum

A

more common

lesion soft, yellow, flat around eyelids

secondary to cholestasis

50
Q

tuberous xanthomas

A

firm, painless, red-yellow nodules

  • extensor of knees, elbows
  • hypercholesterolemia
  • increased LDL
  • secondary to nephrotic or hypothyroidism

resolve after months

51
Q

tendinous xanthomas

A

on tendons

  • related to trauma
  • elevated LDL

years to resolve

52
Q

eruptive xanthomas

A

numerous spread all over body

  • hyper triglyceridemia
  • red-yellow papules
  • spontaneous resolve

with diabetes

53
Q

plane xanthomas

A

dysbetalipoproteinemia

  • occur at any site
  • large areas of face, neck, thorax
  • secondary to cholestasis
54
Q

HDL-C

A

scavenger for lower serum cholesterol

55
Q

LDL-C

A

transport of cholesterol from liver to peripheral tissue

56
Q

severe atherosclerosis

A

hypothyroidism
DM
lipid necrosis

57
Q

niacin

A

vit B3

inhibit release of FFAs

58
Q

pellagra

A

with vit B3 deficiency

59
Q

fibrates

A

decrease secretion of TAG and VLDL

60
Q

ezetimibe

A

inhibit absorption of cholesterol by intestine

61
Q

resins

A

bile acid sequestrants

-bind bile acids - promote excretion in stool