Collagen Biochem and Matrix Disease Flashcards
how much of the ECM makes up our body composition?
80%
what is the ECM?
cellular component of tissues
what does the ECM do?
-provides a scaffold for tissue development
-provides mechanical basis for cell attachment and movement
-transmits force
-withstand compression in cartilage and IVD
-provides survival signals to cells
-reservoir for growth factors
what makes up the ECM?
-water (50%)
-proteins
-glycoproteins
-proteoglycans
-GAG’s
what is the structure of collagen?
-30% of protein mass
-major component of connective tissue
-Gly-X-Y repeat
-triple helical structure
-tesnile forces
what do most common collagen disease mutate?
glycine
what is the function of facit collagen?
regulate fibril diameter, modify fibril interactions
what is the function of network forming collagen?
molecular sieves, planar support for cells
what is the function of transmembrane collagen?
cell matrix interactions, planar support for cells
what is the function of endostatin producing collagen?
produce regulators of cell migration and signalling
what is the function of anchoring fibril collagen?
cell matrix interactions
what is the function of bead forming collagen?
elasticity, long range connectivity
what is the structure of a collagen triple helix?
-3 poly peptide chains
-each chain Is a polypro line helix
-three chains fold into a triple helix
-each chain has a repeating glycine-X-Y residue triplet
what is the process of the collagen maturation?
-pro alpha chains come together in the ER
-proline residues get modified becoming hydroxylated
-this helps protein fold
-lysines are also hydroxylated, becoming a substrate for sugar molecules to attach to
-once triple helix is formed, amino acid no longer available and get secreted
what enzymes cleave the end C/N pro peptides?
C-proteinase
Bmp1 (tolloid family)
N-proteinase
ADAMTS family members
(a disinterring and metalloproteinase with thrombospondin repeats)