Biochem9

Question 1

What is the most abundant protein in the human body?

Answer 1

Collagen

Be (So Totally) Cool, Read Books

Question 2

What is the most common type of collagen?

Answer 2

Type I

Question 3

Where do you find type I collagen?

Answer 3

Bone, Skin, Tendon (Be So Totally)

“Type I: bONE”

dentin, fascia, cornea, late wound repair

Question 4

Where do you find type II collagen?

Answer 4

Cartilage (including hyaline)

“Type II: carTWOlage”

vitreous body, nucleus pulposus

Question 5

Where do you find type III collagen?

Answer 5

Reticulin – skin, blood vessels, uterus, fetal tissue, granulation tissue

Question 6

Where do you find type IV collagen?

Answer 6

Basement membrane or basal lamina

“Type IV: under the FLOOR”

Question 7

A defect in type I collagen results in what?

Answer 7

Osteogenesis Imperfecta

Question 8

A defect in type III collagen results in what?

Answer 8

Ehlers-Danlos

Question 9

Alport syndrome is a result in what collagen type?

Answer 9

Type IV

Question 10

A vitamin C deficiency leads to what?

Answer 10

sCurvy

Question 11

Inability to form triple helix structure during glycosylation results in what?

Answer 11

Osteogenesis Imperfecta

Question 12

What is the most common form of osteogenesis imperfecta?

Answer 12

Autosomal Dominant w/ abnormal type I collagen

Question 13

What are the findings in Osteogenesis imperfecta? (4)

Answer 13

Fractures w/ little trauma (NOT child abuse!!!)
Blue sclerae (d/t translucency of CT over choroidal veins)
Hearing loss (abnormal MIDDLE ear bones)
Dental problems (lack of Dentin)

Question 14

What is the syndrome associated with faulty collagen snthesis causing hyperextensible skin tendency to bleed and hypermobile joints?

Answer 14

Ehlers-Danlos syndrome

Question 15

What significant issues are often associated with Ehlers-Danlos?

Answer 15

Berry Aneurysms
Organ Rupture
Joint dislocation

Question 16

In severe Ehlers-Danlos, which collagen types are most frequently affected?

Answer 16

Type I & Type V

Question 17

Alport syndrome is the result of gene defects in what type of collagen?

Answer 17

type IV

Question 18

What is the most common inheritance of Alport syndrome?

Answer 18

X-linked Recessive

Question 19

What are the findings in Alport syndrome?

Answer 19

Progressive hereditary Nephritis & Deafness

Ocular disturbances

Question 20

What type of collagen is an important structural component of the basement membrane of the Kidneys, Ears, and Eyes?

Answer 20

Type IV

Question 21

Elastin is rich in which AAs?

Answer 21

Proline & Glycine, nonhydroxylated forms

Question 22

What is the defect in Marfan’s syndrome?

Answer 22

Fibrillin

Question 23

What is the pathology of Emphysema?

Answer 23

Alpha1-antitrypsin defciency –> INcreased elastase activity