Fibrous Proteins- Keagan (week 3) Flashcards
Structure of collagen
Right-handed triple helix of alpha-chains
Most abundant protein in the body
What are alpha chains and their repeats
Individual poly peptides composing tropocollagen
GLY-X-Y repeats
Proline and hydroxyproline/hydroxylysine are usually X and Y
Three polypeptide alpha chains of collagen held together by
Hydrogen bonds
Type I collagen
Composition
Tissues
Genes
Composition- a1[I], a2[I]
Tissues- skin, bone, cornea, tendons
Genes- COL1A1, COL1A2
Type II collagen
Composition
Tissues
Genes
Composition- a1[II]
Tissues- cartilage, intervertebral disks, vitreous body
Genes- COL2A1
Type III collagen
Composition
Tissues
Genes
Composition- a1[III]
Tissues- blood vessels, dermis
Genes- COL3A1
Type IV collagen
Composition
Tissues
Genes
Composition a1, a2, a3, a4, a5, a6
Tissues- basement membranes
Genes- COL4A1,2,3,4,5,6
Type V collagen
Composition
Tissues
Genes
Composition- a1 or a1[V]a2[V]
Tissues- interstitial tissue (associated with type I)
Genes- COL5A1,2,3
Collagen made by what three cells
Osteoblasts
Chondroblasts
Fibroblasts
Biosynthesis of collagen
Idk watch video or something
Vitamin C importance
Deficiency causes what
Reducing agent (coenzyme) to hydroxylate PRO and LYS
PRO—> PRO-OH
Deficiency causes scurvy
Osteogenesis imperfecta
Type I collagen disorder (mutation of type I collagen)
-brittle bone disease, fractures, blue sclera, hearing loss
Ehlers-danlos syndrome
Hyperelasticity of skin, hyper extensive joints, rupture of colon, skin hemorrhage
Mutations of type I, III, V collagen
Menkes Disease
X-linked recessive
Deficient lysyl oxidase (cross linking)
Copper deficiency
Synthesis of elastin
Tropoelastin + fibrillin-1 (microfibrillar proteins)