COLLAGEN Flashcards
Primary structure of collagen
Repeated sequence: (Gly-X-Y)n
X,Y: mostly Pro and HyPro
Secondary structure of collagen
left handed helical strycture of 1 poly peptide
Procollagen + stabalizing forces
A right handed triple helix with globular parts at the ends so cant polymeize
from 3 left handed collagen helix
1. Interchain H- bonds
between peptide groups
2. Cross links between 2 Lys ( no Cys)
Function of
globular parts :
- Promote folding
2. Inhibit premature fiber formation
Tropocollagen:
the globular parts at the ends are cut
the globular parts at the ends are cut by Procollagene peptidases
Abnormalities of collagen formation
Inherited diseases:
Osteogenesis Imperfecta (fragile bones) Procollagen (triple helix) formation is incomplete
Ehler-Danlos syndrome (strechable skin, hypermobyl joints)
Decreased activity of procollagen peptidase
Aquired disease:
Menkes syndrome (kinky hair, growth retardation): A dietary deficiency of copper ion needed for lysyl oxidase (lys-lys covalent cross link formation)