Fats

Question 1

propionyl Coa source

Answer 1

odd chain fa and 4 aa

Question 2

carboxylase enzymes

Answer 2

1. gluconeogenesis-pyruvate carboxylase
2. fa synthesisi -acetyl co carboxylase
3. odd chain meta -propionyl coA carboxylase

Question 3

methylmalonic aciduria

Answer 3

1. b12 def.
2. IF defect
3. methlymalonyl CoAMutase
4. defect in conversion to coenzyme form of b12

Question 4

methylmalonic aciduria diff which 2 conditions

Answer 4

def. of b12 and b9

Question 5

why b12 def leads to peripheral neuropathy

Answer 5

propionly coa and methlymalonyl coa builds up in cells so does in myelin

Question 6

only 1 organ make ketone bodies

Answer 6

liver mitochondria

Question 7

hmg CoA reductase enz fxn and types

Answer 7

1. cholesterol syn-in ER of all cells of body ex RBC

2. ketone body- ONLY in liver mito

Question 8

name ketone bodies

Answer 8

acetoacetate
b-hydroxybutyrate
acetone

Question 9

if there is no hormonal control of ketone bodies synthesis then how is it reg?

Answer 9

acetylCoa1.krebs cycle
2.ketone body
during starvation OAA is uesd for glucobeogenesis so krebs cycle cant run.
only option left is kb formation

Question 10

how much atp from acetoacetate

Answer 10

24 atp.(b-hydroxybutyric acid generate 1 mol of nadh also and gets converted to acetoacetate)

Question 11

kb formation and utilization

Answer 11

mito

Question 12

thiophrase significance

Answer 12

used in ketone bodies oxidation.absent in liver so it cant use kb

Question 13

during starvation OAA in liver for gluconeogenesis so acetylcoa cant be used..wat is the state in other tissues

Answer 13

kb -acetlycoA-krebs cycle bcoz no gluconeogenesis

Question 14

ketosis

Answer 14

1. ketonemia -acidosis
2. ketoneuria-draws water also,dehydration
3. acetone breath

Question 15

which of the ketone cant be used as fuel

Answer 15

acetone

Question 16

brain fuel use

Answer 16

1/2d glycogen
1/2 d gluconeogenesis peak
till 1w gluconeogenesis -mostly muscle protein
after 1 wk: slows glucose use and use more ketone-from adipose
also gluconeogenesis from liver and kidney forblood levels

Question 17

fxn of sugars attached to lipids/proteins

Answer 17

blood group
cell cell recognition
act as receptors-cholera toxin Gm1 ganglioside receptor

Question 18

shingolipids aka

Answer 18

glycolipids

Question 19

sphingolipid mainly found in

Answer 19

plasma memb

Question 20

ganglioside

Answer 20

sialic acid attached to glycolipid

eg:N acetyeuramic acid nana

Question 21

n acetyl galactosamine

Answer 21

a sugar of hexosamine class
hexosaminidase A def. in tay sachs(cherry red spots)

Question 22

cherry red spot

Answer 22

tay sachs

neimann pick

Question 23

gaucher

Answer 23

glucocerebrosidase def. in lysosome

crumpled/wrinkled paper inclusions

Question 24

eg of ds treated with enz replacement

Answer 24

gauchers ds

Question 25

sphingomyelinase def leads to

Answer 25

neimann pick-cherry red spots foamy macrophages zebra bodies

Question 26

i cell ds

Answer 26

defect in phosphotransferase in golgi. all lysosomal enz absent inclusion bodies

Question 27

inlclusion bodies

Answer 27

sphingolipidosis | i cell ds