COD Chondrodysplasias

Question 1

What is skeletal dysplasia? (osteochondrodysplasia)

Answer 1

Clinically and genetically heterogeneous disorders of bone and/or cartilage.
characterized by abnormalities in patterning, linear growth, differentiation, and maintenance of the human skeleton

Question 2

Describe endochondral ossification

Answer 2

Is the one of 2 essential processes during fetal development of the mammalian skeletal system

1) Cartilage formation
- mesenchymal cells divide and differentiate into chondrocytes
- secrete cartilage
- become embedded in lacunae within matrix
2) Vascular invasion and longitudinal growth
- ring of woven bone formed in midshaft
- osteoclasts allow vascular invasion of woven bone and cartilage
- growth plate forms

Question 3

How does the growth plate regulate bone length?

Answer 3

Long bones continue to lengthen through the addition of bone tissue at the epiphyseal plate.

Question 4

Describe the molecular organisation of cartilage matrix

Answer 4

Formed as chains
Twists into triple helices
They are lined up and bonded together into fibrils
These fibrils are arranged in layers