Loading....
Coupon Accepted Successfully!

Orthopaedic

Open Flashcards

Metabolic Bone Diseases

Question
23 out of 42
 

The disease that results in deficient hydroxyla¬tion of the pro-alpha chains of collagen is known as



A Scurvy
B Rickets

C Ehlers-Danlos syndrome
D Marfan's syndrome

Ans. A

Scurvy

a. Proline hydroxylase and lysine hydroxylase are responsible for hydroxylation of the pro-alpha chains of collagen in the endoplasmic reticulum.

b. These enzymes require fer­rous iron, molecular oxygen, and ascorbic acid (vitamin C. as cofactors. Scurvy, or vitamin C deficiency, impairs the function of these enzymes.

c. Patients with Ehlers-Danlos syndrome have defects in the cross-link­ing of mature collagen fibers. Marfan's syndrome has many variants, most of which exhibit extra amino acids on the procollagen chains.

d. Lathyrism is a disease of animals caused by eating the seeds of sweet peas, which contain a substance that inhibits cross-linking of mature collagen fibers

Metabolic Bone Diseases Flashcard List

42 flashcards
1)
2)
3)
4)
5)
6)
7)
8)
9)
10)
11)
12)
13)
14)
15)
16)
17)
18)
19)
20)
21)
22)
23)
24)
25)
26)
27)
28)
29)
30)
31)
32)
33)
34)
35)
36)
37)
38)
39)
40)
41)
42)