Carb Metabolism 5 Flashcards Preview

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Flashcards in Carb Metabolism 5 Deck (11)
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1
Q

GAGS have what type of charge, which attracts what?

A

negative charges due to sulfates; attachs cations

2
Q

What are 6 proteoglycans?

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

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

A

rER

aspargine and serine: golfi

4
Q

What are finished proteoglycan molecues linked to?

A

hyaluronic acid

5
Q

Where are proteoglycans degraded?

A

lysosomes

6
Q

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

A

mucupolysaccharidosis (MPS)

7
Q

What are three types of mucopolysaccharidoes?

A

Hunters: Dermatan sulfate
Heparan sulfate

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

8
Q

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

A

mannose, galactose, and fucose

9
Q

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

A

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

10
Q

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

A

p450 cytochrome to NABQI

11
Q

What is Crigler-Najjar Syndrome?

A

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