ECM and adhesion Flashcards
what is a complex network of secreted macromolecules
ECM
what does GAG stand for
glucosaminoglycans
the ECM provides a 3D framework for
organisation of tissues and basic cellular processes
the ECM regulates things
proliferation, differentiation, migration, cell-cell interaction
composition of ECM macromolecular network
collagens, elastin, glycoproteins, proteoglycans
example of ECM disease- too much ECM
fibrosis
how much of total protein in body is collagen
25%
what are the primary structural components in connective tissue
collagens
how many different types of collagens have been identified
> 25
what is the structure of collagen and amino acid sequence
triple helical structure Gly-X-Y ie. every 3rd amino acid is glycine
2 main categories of collagen
fibrillar (fibril forming, well organised banded fibrils) and non-fibrillary (form microfibrils/ mesh like structures)
type I and II are what type of collagen
fibrillar
type IV and VI are what type of collagen
non-fibrillary
types I and II are major components in ? and provide ?
tendons, ligaments, skin- provide high tensile strength
type IV and VI are structural components of ? and provide ?
basement membranes- provide anchorage of cells and filtration
what is the name for brittle bone disorder
osteogenesis imperfecta
osteogenesis is caused by genetic defects in which type of collagen
collagen I
what are 5 symptoms of OI
fragile bones, think skin, abnormal teeth, think sclera, weak tendons
what amino acid is mutated in OI
glycine to a more bulky one
OI is what type of mutation
single base
which type of OI is most common I or II
type I (50%)
Type I OI is autosomal dominant/ recessive
dominant
which type of OI is the mildest
Type I
which genes are mutated in OI type I
COL1A1 and A2 genes on chromosomes 7 and 17
which type of OI is more severe
type II