CT

Collagen

• nonfibrillar collagen

Alport

Collagen Synthesis

Scurvy

• gums

• propeptide added to fibrillar collagens

Pathology

Osteogenesis

Ehlers Danlos

• classic type

Menke

• opposite of wilson

Elastin

AAT

Williams

• elastin important component of aorta

Fibrillin

Marfan

• CT abnormal, allow pts to grow very tall with long arms

• spontaneous pneumothorax

• weak aortas because weak elastin from fibrillin deficiency