Osteogenesis imperfecta

1. Osteogenesis imperfecta is a bone disorder that arises from an inability to form

________ (problems forming a triple helix of collagen alpha chains)
a. Collagen 1
b. Procollagen
c. Fibroblasts

2. What is the genetic inheritance of the most common form of Osteogenesis imperfecta?
a. autosomal recessive
b. autosomal dominant
c. x-linked

3. ______ is a complication of Osteogenesis imperfecta that results from abnosrmal ossicle formation.
a. Ehlers Danlos syndrome
b. Hearing loss
c. Brittle bone disease

4. Dental imperfections are a complication of Osteogenesis imperfecta due to a lack of ____ formation.
a. Collagen 1
b. Colagen 11
c. Dentin

5. What type of collagen is found in bone?
a. Type 2
b. Type 1
c. Type 3

5. What type of collagen is found in hyaline cartilage found in articulating surfaces?
a. Type 2 collagen
b. Type 1 collagen
c. Type 3 collagen

6. What type of collagen is abundant at the glomerular baseline?
a. Type 2 collagen
b. Type 1 collagen
c. Type 3 collagen
d. Type 4 collagen
e. Type 5 collagen

7. What type of collagen is found in scars?
a. Type 2 collagen
b. Type 1 collagen
c. Type 3 collagen
(Type 1 collagen replaces type 3 collagen in scar formation)

8. What trace element that acts as a cofatcor for Lysyl Oxidase.
(Lysyl Oxidase is an enzyme which cross-links collagen)
a. Copper
b. Zinc
c. Lead
d. Magnesium

9. What is the most abundant protein in the human body?
a. Albumin
b. collagen

10. What type of cells make collagen?
a. osteoclasts
b. osteoblasts

11. What type of collagen is found in tendons?
a. Type 2 collagen
b. Type 1 collagen
c. Type 3 collagen

12. What type of collagen is found in the cornea?
a. Type 2 collagen
b. Type 1 collagen
c. Type 3 collagen

13. What type of collagen is found in dentin?
a. Type 2 collagen
b. Type 1 collagen
c. Type 3 collagen

14. What type of collagen is found in reticulin?
a. Type 2 collagen
b. Type 1 collagen
c. Type 3 collagen

15. What type of collagen is found in blood vessel walls?
a. Type 2 collagen
b. Type 1 collagen
c. Type 3 collagen (as reticulin)

16. What type of collagen is found in granulation tissue?
a. Type 2 collagen
b. Type 1 collagen
c. Type 3 collagen (as reticulin)

17. What type of collagen is deficient in the vascular type of
Ehlers-Danlos Syndrome?
a. Type 2 collagen
b. Type 1 collagen
c. Type 3 collagen

18. What type of collagen is found in the lens of the eye?
a. Type 2 collagen
b. Type 1 collagen
c. Type 4 collagen

19. Collagen is made by first making a three-dimensional stranded structure called procollagen. What amino acids are needed to make procollagen?
a. proline, lysine
b. glycine, lysine
c. glycine, proline

20. On the way to becoming collagen, procollagen is modified by the addition of hydroxyl groups to the amino acids, ___ and ____. This step is important for glycosylation and formation of the triple helix structure of collagen.
a. proline, lysine
b. glycine, lysine
c. glycine, proline

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