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