Pathophys - Cartilage and Bone Flashcards
mesenchymal stem cell derivatives
- muscle cells
- adipocytes
- osteoblasts
- fibroblasts
- chondroblasts
what is unique about the derivation of osteoclasts?
they come from the monocyte cell line
fibroblasts secrete what?
components of connective tissue
define connective tissue
- tissue that connect, supports, binds, or separates other tissues or organs
- typically have relatively few cells embedded in an amorphous matrix
- often has collagen or other fibers like cartilagenous, fatty and elastic tissues
what is the most abundant protein in the body?
collagen
proteoglycans are precursor molecules for what?
collagen
function of proteoglycans
bind collagen fibrils, elastic fibers and reticular fibers together
where do elastic fibers predominate?
in blood vessels and ligaments
reticular fibers predominate where?
in lymphatic tissue and basement membranes
vitamin C is a cofactor for what process?
hydroxylation of proline and lysine
the deficiency of vitamin C causes what?
- scurvy
- poor wound healing
ehlers-danlos syndrome
- defective procollagen peptidase results in uncleaved procollagen or in lysyls hydroxylase
- so can’t hydroxylate lysine needed for staggered array of tropocollagen
- hyperelasticity, joint dislocations, vascular wall rupture
osteogenesis imperfecta
- point mutation in collagen gene resulting in reduction in type 1 collagen
- type 1 collagen is needed for normal ossification
- brittle bones and blue sclera
what are the two most important things to remember about osteogenesis imperfecta?
- type 1 collagen deficient
* blue sclera
goodpasture’s syndrome
- auto-antibodies to type IV collagen
- results in renal and or lung basement membrane thickening
marfans syndrome
- defect in fibrillin 1 gene, chromosome 15
- fibrillin surrounds elastic fiber core
- results in dilation of aorta, mitral prolapse, periosteal changes (elongation)
presentation of pt w/ marfan’s
- tall stature
- high arched palate
- myopia
- mitral valve prolapse
a mitral valve prolapse causes what heart sound?
systolic click
general overview of collagen production (in terms of cells)
- chondroblasts form chondrocytes
- chondrocytes make tropocollagen
- tropocollagen molecules overlap into collagen fibrils
- fibril bind together to form fibers
- fibers bind together to form tendons
structure of tropocollagen
3 alpha strand proteins arranged in alpha helix
functions of ECM
- cell adhesion
- cell-cell communication
- differentiation
ECM formation
- chondroblast form chondrocytes
- chondrocytes secrete ECM
- young collagen from ECM matures
ECM is primarily made of what two things?
- aggregan
- type II collagen