ECM High Yield

Question 1

What are glycosaminoglycans made of?

Answer 1

repeating disaccharide units

Question 2

What are the seven glucosaminoglycans that differ in the monosaccharides present in the repeating disaccharide units?

Answer 2

chondroitin sulfate, dermatan sulfate, heparin, heparin sulfate, hyaluronic acid, and keratin sulfate I and II

Question 3

Where does the synthesis of proteoglycan structure occur?

Answer 3

the ER

Question 4

Where are the carbohydrate monomers added to the protein? (Glycoproteins)

Answer 4

in the lumen of the ER and the Golgi complex

Question 5

How often does a Glycine residue occur in the triple helical portion of the alpha chain?

Answer 5

every third position

Question 6

What is collagen synthesized as?

Answer 6

preprocollagen

Question 7

What type of chain and composition does the basement membrane have?

Answer 7

Type 4 collagen, composition [a1(IV)]2a2(IV)

Question 8

Ehler-Danlos Syndrome

Answer 8

• hyerextensibility of the skin, abnormal tissue fragility, increased joint mobility
• type 4 Ehler-Danlos is the most serious (spontaneous rupture of arteries of the bowel reflecting abnormalities in type 3 collagen)
• type 6 due to lysyl hydroxylase deficiency (joint hypermobility and ocular rupture)
• type 7c due to procollagen N-proteinase deficiency (abnormal thin, irregular collagen fibrils (joint hypermobility)

Question 9

Alport syndrome

Answer 9

affecting the strucutre of type 4 collagen fibers (BASEMENT MEMBRANE)

Question 10

What is a genetic disease of Elastin?

Answer 10

Williams syndrome (affecting connective tissue and CNS)

Question 11

Marfan syndrome?

Answer 11

autosomal dominant, affecting connective tissue, due to mutations in fibrillin gene