Carb Metabolism 5

Question 1

GAGS have what type of charge, which attracts what?

Answer 1

negative charges due to sulfates; attachs cations

Question 2

What are 6 proteoglycans?

Answer 2

1. chondriten sulfate
2. heparin sulfate
3. dermatin sulfate
4. keratin sulfate
5. heparin
6. hyaloronic acid

Question 3

Where is the protein moeity of proteoglycans synthesized? Where are the sugar residues are added to what residues iwhre?

Answer 3

rER

aspargine and serine: golfi

Question 4

What are finished proteoglycan molecues linked to?

Answer 4

hyaluronic acid

Question 5

Where are proteoglycans degraded?

Answer 5

lysosomes

Question 6

If tehre is a defective proteoglycan-degradign hydrolase, substrates will accumulate in the lysosomes, results in what types of diseases?

Answer 6

mucupolysaccharidosis (MPS)

Question 7

What are three types of mucopolysaccharidoes?

Answer 7

Hunters: Dermatan sulfate
Heparan sulfate

Hurler Scheie: dermatan sulfate; hepara sulte
Sanfilippos : hep sul

Question 8

For the glycosylation of proteins, the cell needs to synthesize what 3 things?

Answer 8

mannose, galactose, and fucose

Question 9

What is the UGT reaction where the body can excrete poorly water-soluble molecules such as hemoglobin, steroid hormones and xebobiotics? (1 step). Use aspirin in this example

Answer 9

UDP-glucuronate + acetaminophen –> UGT–> UDP + x-glucuronate (less hydrophobic)

Question 10

If the UDP-glucuronylation pathway of detox is overwhelm, acetaminophen is oxidized by what?

Answer 10

p450 cytochrome to NABQI

Question 11

What is Crigler-Najjar Syndrome?

Answer 11

defect in UGT, so you can make is more water soluble; a buildup of unconjugated bilirubin leads to jaundice