L15 Flashcards
What does the ECM contain ?
structural proteins : collagen , elastin , fibrin-1
specialized proteins : fibronectin , laminin
proteoglycans
carbohydrates in glycoproteins attach to ___
the side chain oxygen of amino acid serine or threonine by O-glycosidic bonds
the side chain of N of Asparagine residue by N-glycosidic linkages
glycosaminoglycans are also known as mucopolysaccharides and they are ____
hetropolysachharide
where are glycosaminoglycans present ?
extra cellular matrix and connective tissue
glycosaminoglycans contain repeating units of __
amino sugars and uronic acids
they may also contain negatively charged Sulphate or carboxylate group
Glycosaminoglycans , when found in association with proteins are called ____
proteoglycans
what is mucopolysaccharidoses ?
genetic disorders characterized by deficiencies of enzymes that degrade glycosaminoglycans resulting in accumulation
Examples: Hurler syndrome, Hunter syndrome
degration of glycosaminoglycans is carried out by _____
lysosomal hydrolyses
where are the following types of collagen found : TYPE 1: TYPE 2 : TYPE3: TYPE 4: TYPE 8:
type 1 : bone , ligaments , scar tissue , tendons
type 2:hyaline cartilage
type 3: blood vessels , skin , muscle
type 4: lens of eye , basal lamina beneath epithelium , capillaries
type 8: corneas and vascular endothelium
type 4 and 8 collagen are network forming
..
what are collagenopathies ?
number of genetic diseases that result from abnormalities in the synthesis of collagen .
what is fibrillin-1 ?
large glycoprotein that is secreted into matrix by fibrocblasts .
fibrillin-1 becomes incorporated into microfibers , which ____
provide a scaffold for deposition of elastin
where can fibrillin-1 be found ?
zonular fibers of the lens , in the periosteum and associated with fibers in the aorta
what does matrons syndrome result in ?
abnormal fibrillin-1 and/or lower ammonites being deposited in ECM
