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17 Cards in this Set

  • Front
  • Back

Collagen is made up of?

3 alpha polypeptide chains

Collagen type 1 contains what chains?

2 (alpha1 chains)


1 (alpha 2 chain)collagen type 2

Collagen type 2 has what chains?

3 (alpha1 chains)

Type 1 collagen found in?

Bones


Tendons


Blood vessels


Cornea of eye

Type 2 collagen is found in ?

Cartilage

Type 3 collagen is found in?

Skin and blood vessels

Gly-X-Y



What amino acids frequently occupies the X position and Y position in collagen peptide chains?

X = Proline


Y = 1. hydroxyproline


2. Hydroxylysine

Hydroxyproline's contribution to collagens tensile strength?

Forms hydrogen bonds between collagen alpha chains to stabilize triple helical structure

Hydroxyl group of Hydroxylysine is involved in?

Enzymatic glycosylation of collagen

What co factor is needed for enzymatic hydroxylation of proline and lysine to form Hydroxylysine and hydroxyproline

Vitamin C (Asorbic acid)


Fe++


O2

Where in the cell does the pro alpha chain undergo hydroxylation?

RER


Rough endoplasmic reticulum

The cleavage of pro-collagen N terminus and C terminus ends by procollagen peptidases occurs...................... And releases.......................

1.In the extra cellular space


2. Triple helical tropocollagen

Cross linking of individual collagen fibrils yields mature collagen fibers. How is this cross linking derived?

Deamination of lysine and Hydroxylysine by copper containing enzyme Lysyl Oxidase to form reactive aldehydes Allysine & Hydroxallysine


These form covalent bonds with lysine and hydroxy lysine of another collagen fibril.

Ehlers-Danlos Syndrome causes and types?

Deficiency in enzymes Lysyl hydroxylase & procollagen peptidases.



Mutation in A.A sequence of collagen type 1,2,3



Classic EDS = Collagen type V defect.



Vascular EDS = Defect in collagen type 3

Osteogenesis Imperfecta causes and types?

Defect in collagen type 1 (mutation where Glycine is replaced by a bulky A.A in the collagen alpha chain)



Type1 = most common (mild bone fragility, hearing loss, blue sclera)



Type2 = in utero fractures seen in fetus.



Type3 = severe. Multiple fractures at birth, kyphosis, blue sclera, short stature.

Children with osteogenesis Imperfecta can be treated with?

Bisphosphonates


(Inhibits osteoclastic activity)

Elastin's properties are?

Elastic in nature and insoluble