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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

glucagon

A

does not stimulate lipid release

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

insulin

A

stimulate lipid release

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

increased hormone sensitive lipase

A

TGL > glycerol + FAs

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

fatty acids

A

adipose tissue

  • carried with albumin in blood
  • to liver

-beta oxidation to acetyl CoA**

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

acetyl CoA

A

can go to ketone bodies
-brain and cardiac muscle

or to citric acid cycle

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

alpha oxidation

A

peroxisomes

-phytanic acid breakdown

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

omega oxidation

A

in some species of animals

alternate path to beta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

beta-oxidation

A

long chain FA breakdown

in mito to produce acetyl CoA

to TCA cycle - to ETC > ATP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

short chain FAs

A

2-4C

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

medium chain FAs

A

6-12C

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

short and medium chain FAs

A

diffuse freely into mitochondria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

long chain FAs

A

14-20C

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

very long chain FAs

A

> 20C

  • enter peroxisomes
  • unknown mechanisms
  • oxidation occurs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

CPT1

A

outer mito membrane
-fatty acyl CoA to fatty acylcarnitine

-then transported across inner mito membrane

aka CAT1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

CPT II

A

inner mito membrane

fatty acylcarnitine converted back to fatty acyl CoA in mitochondrial matrix

aka CAT2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

fatty acyl CoA synthetase

A

FA + CoA > fattyl acyl CoA

outer mito membrane

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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**

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

MCAD

A

medium chain acyl-CoA DH deficiency

fasting hypoglycemia
no ketone bodies
vomiting
coma, death

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

carnitine

A

can be measured in blood

if present in blood - either CPT1 or CPT2 deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

CPT1 and 2 deficiency treatment

A

avoid fasting
dietary restric long chain FAs
carnitine supplement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

muscle weakness with exercise, hypoerammonemia, death

A

CPT II deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

fasting hypoglycemia, inability to use LCFAs as fuel by liver

A

CPT I defiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
medium chain FAs
do not require carnitine shuttle
26
liver disorder
likely more involvement of carnitine deficiency than CPT1 or CPT2
27
causes of carnitine deficiency
- 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
Tx of carnitine deficiency
pharm carnitine oral dose avoid fasting/exercise supp medium chain FA high carb, low fat diet
29
browin urine, pain in arms and leg muscle when exercise, recommended carnitine supplement - likely diagnosis?
CPT II deficiency
30
CPT I deficiency
with liver dysfunction
31
MCAD
usually first 3-5 years of life
32
tall stature - long bones
marfan
33
brown urine and muscle weakness
CPT II deficiency
34
xanthomas
accumulation of lipid-laden macrophages from altered lipid metabolism or local cell dysfunction
35
hyperlipoproteinemia
cause of xanthomas | -primary and secondary
36
primary hyperlipoproteinemia
5 phenotypes -also decreased synthesis of HDL or hepatic lipase deficiency
37
type I hyperlipidemia
familial lipoprotein lipase deficiency - accumulation of chylomicrons - early childhood - acute pancreatitis - eruptive xanthomas - elevated plasma triglycerides
38
type III hyperlipidemia
familial dysbeta-lipoproteinemia - accumulation of IDL - both triglyceride and cholesterol increase - mutation of apo E premature atherosclerosis and xanthomas
39
type IV hyperlipidemia
familial hypertriglyceridemia - over-production of VLDL - elevated plasma triglyceride levels - cholesterol low eruptive xanthomas coronary heart disease DM II, obesity, alcoholism
40
type IIa hyperlipidemia
accumulation of LDL - LDL receptor deficiency - defective apo B100 - elevated plasma cholesterol - normal plasma triglyceride - severe atherosclerosis **tendinous xanthomas, tuberous xanthomas, xanthelasmas
41
type IIb hyperlipidemia
accumulation of LDL and VLDL - defective apoB100 - variable levels of triglyceries and cholesterol tendinous xanthomas, tuberous xanthomas, xanthelasma
42
apo B100
for storage in adipose tissue
43
apo B48
chylomicron dietary lipid
44
apoproteins
synthesized in liver -mixed with lacteals (chylomicrons) in heart -with protein on outside
45
type V hyperlipidemia
genetic defect in apo-lipoprotein CII - accumulation of chylomicrons and VLDL - elevated triglyceride - early childhood - acute pancreatitis - eruptive xanthomas
46
decreased synthesis of HDL
decrased apo A1 and apo CII decreased reversed cholesterol transport increased LDL premature CAD plane xanthomas
47
hepatic lipase deficiency
accumulation of large triacyl-glycerol rich HDL and VLDL coronary heart disease xanthomas
48
cutaneous xanthomas
with hyperlipidemia
49
xanthelesma palpebrarum
more common lesion soft, yellow, flat around eyelids secondary to cholestasis
50
tuberous xanthomas
firm, painless, red-yellow nodules - extensor of knees, elbows - hypercholesterolemia - increased LDL - secondary to nephrotic or hypothyroidism resolve after months
51
tendinous xanthomas
on tendons - related to trauma - elevated LDL years to resolve
52
eruptive xanthomas
numerous spread all over body - hyper triglyceridemia - red-yellow papules - spontaneous resolve with diabetes
53
plane xanthomas
dysbetalipoproteinemia - occur at any site - large areas of face, neck, thorax - secondary to cholestasis
54
HDL-C
scavenger for lower serum cholesterol
55
LDL-C
transport of cholesterol from liver to peripheral tissue
56
severe atherosclerosis
hypothyroidism DM lipid necrosis
57
niacin
vit B3 inhibit release of FFAs
58
pellagra
with vit B3 deficiency
59
fibrates
decrease secretion of TAG and VLDL
60
ezetimibe
inhibit absorption of cholesterol by intestine
61
resins
bile acid sequestrants | -bind bile acids - promote excretion in stool