cell matrix Flashcards
exam 2
connective tissue matrix components (4)
elastic fiber
proteoglycan
hyaluronan
Type I collagen
Basement membrane is the same as the
Basal Lamina
thin protein membrane based structure
Basement membrane
sugars on the proteoglycan create
a strong barrier
genetic defect causes deficinecy in copper utilization and thus faulty collagen crosslinking
Menke disease
Genetic defect in which Type IV collagen is absent or non-functional.
Alport syndrom
Collagen IV commonly found in
Basement membrane
second most common inherited PKD
Alport syndrome
autoimmunie disease in which antibodies to Type IV collagen destroy the basement membrane in the lung and kidneys
Goodpasture syndrome
20 kd fragment of Collagen XVIII
blood vessel-specific endostatin
endostatin is cleaved from
Collagen XVIII by an MMP
used in 4 million patients; endostatin, angiostatin and anti-VEGF antibody
angiogenesis
endostatin specificity
immature vascular endothelial cells
Endostatin likely mechanism
induces apoptosis in EC
ATP7A gene defect
menke
proteoglycan major function
provide selectively permeable filters in kidney and lung
more protein than carbohydrates and N-linked
glycoproteins
o-linked, huge amount of carbohydrates vs protein
proteogylcans