091614 dermis Flashcards
roles for the ECM of the dermis
supports cells in the dermis
regulates cell fxn
ECM of dermis has
made of collagen, elastic fibers, extrafibrillar matrix
main component of dermis
collagen
most abundant collagen in dermis
collagen I
second most abundant collagen of skin
collagen III
increased in levels during embryogenesis and infancy and in wound healing
what collagens are in the basement membrane of skin
IV, VII, XVII
structure of elastic fibers in dermis
microfibrils (mainly fibrillin)
elastin
ground substance of dermis is composed of
water, electrolytes, plasma proteins, proteoglycans
proteoglycan structure
protein core with GAGs
fxn of proteoglycans
binds lots of water
how is collagen made?
made by fibroblasts
intracellularly, three chains twisted together to form procollagen
extracellularly, procollagen gets cleaved at both termianals. then gets assembled. combined into collagen fibrils
Marfan syndrome
autosomal dominant
mutation in fibrillin, affecting elastic fibers
variable expression
what features can you see w/ Marfan syndrome
ectopia lentis
myopia
pectus excavatum
aortic dilation or aneurysm
mitral valve prolapse
short torso
scoliosis
tall and thin body
long limbs and fingers
flexible joints
striae (skin able to stretch but not able to return)
Ehlers Danlos syndrome
group of inherited connec tissue disorders
abnormalities of collagen struc, production, processing, or assembly
variable inheritance. clinical features based on mutation
skin not as stable as it should be
clinical features of Ehlers danlos
hypermobile joints
skin is very stretchy