Lysyl Hydroxylase

Prolyl Hydorylase

Lysyl Oxidase

Glycosyl transferase

Amino and Carboxy terminal peptidases

Osteogenesis Imperfecta

Ehlers - Danlos

Alport Syndrome (hereditary nephritis)

Menkes

Scurvy

Homocyteinuria

Lathyrism

Describe Lathyrism

Collagen disorder characterized by abnormal cross-linking due to inhibition of lysyl oxidase

Symptoms include deformation of the spine, dislocation joints, deminerlization of bones

Is a dietary disease that arise from regular consumption of Lathyrus Odoratus, Sweet Pea...Contains Beta-aminoproprionitrile which inactivates lysyl oxidase.

Penicillamine is also a cross link inhibitor used in chelation therapy to treat Wilson's Disease (copper overload disease). Chelates copper, making it unavailbale to lysyl oxidase.

alpha 1: hydrolyzes GLy-Ileu

alpha 2: hydrolyzes Gly-Leu

Requires Ca2+ and Zn2+

synthesized as procollagenase (inactive)

involved in growth and remodeling of tissue

found in gangrene 

Cleaves right before --Gly-Pro--

Breaks down connective tissue barrier, allowing the spread of bacteria

A serine protease

Hydrolyzes elasin and other proteins

Does NOT hydrolyze collagen

Alpha 1 antitrypsin: will neutralize elastase, collagenase, and other serine proteases

Alpha 2 macroglobulin: will neutralize elastase and other serine proteases

Emphysema: arises from imbalance of alpha 1 antitrypsin and elastase; cigarette smoke reduces the numbers of alpha 1 antitrypsin

aka mucopolysaccharide

Polyanionic complex that consists of glycosaminoglycans covalently bound to a core protein