- Discussion:
- the
biosynthesis of collagen involves many complex processes that
are subject to biologic control;
- these processes can be disrupted at any point by metabolic disorders such as:
-
scurvy;
-
lathyrism;
-
genetic collagen diseases:
-
Ehlers-Danlos syndrome
- the collagen extracted from pts with type VI Ehlers-Danlos syndrome is known
to be deficient in hydroxylysine;
- type VII Ehlers-Danlos syndrome is now known to be associated w/ a deficiency
of procollagen peptidase;
- once the
osteoblasts secretes procollagen, the patient either has no procollagen
peptidase to remove the amino terminal endpiece or the patient does have
procollagen peptidase, but it is inactive due to defect located within the chain;
-
Osteogenesis Imperfecta;
-
Marfan's syndrome:
Etiology and pathogenesis of heritable connective tissue diseases.
WB Cole.
Journal of Pediatric Orthopaedics.
Vol 13. 1993. p 392-403.
