Fibrous Protein
Collagen
Collagen and Collagen Related Diseases
What is the Pathology?
Pathology?
Pathology?
Pathology?
Mutation Underlying Osteogenesis Imperfecta
Levels of Collagen Structure
Collagen Role in Bone Formation
Levels of Collagen Structure Models
Collagen Primary Structure
Hydroxyproline
Collagen Primary Structure and Interchain Hydrogen Bonding
Model of Tripple Helix
Stabilizing the Triple Helix
Roles of Hydroxyproline
Collagen Synthesis
Steps in Collagen Biosynthesis
Function of Collagen Pro-Peptides
Collagen Pro-Peptides
Collagen Biosynthesis from transcription to formation
Hydroxylation of Lysine
Hydroxylation of Proline
Why is Ascorbate acid essential in Collagen Biosynthesis
Role of Hydroxyproline
Lysine and Proline Hydroxylases
Scurvy
Cross-Link Formation in Collagen
Post translational modification in lysine crosslink formation
One type of Cross-Link Seen in Collagen
Collagen Disorders
Elastin
What is Elastin
Major differences between Collagen and Elastin
Williams Syndrome and Supravalvular Aortic Stenosis
Elastin Primary Structure
Elastin Biosynthesis from transcription to formation
Entropy Driven Elastic Recoil
Elastin Open and Closed State
Elastin Stretching the Limits
Desmosine
Building Elastin
Fibrillin and Fibulin
Fibrillin 1 Secretion
Marfan’s Syndrome
Conclusion