Excellent new review from Patrick Yap and Ravi Savarirayan discusses emerging targeted drug therapies in :-
- Osteogenesis Imperfecta (brittle bone disease)
- Other Emerging Skeletal Dysplasia Therapies
And don’t forget our own review from last year……..
Very interesting indeed………first missense mutations identified in Biglycan, a member of the class I family of small leucine-rich proteoglycans (SLRPs) that is found in the extracellular matrix of bone and cartilage………
Could this be another example of a protein misfolding – ER stress related human connective disease……..?
See here for full article
Targeted mouse models of recessive spondyloepimetaphyeal dysplasia, aggrecan type
Why Make Mouse Models of the Aggrecanopathies?
Because cell model studies to date have provided no links between aggrecan mutations and disease mechanisms…….
Image from ANIMAL INSIDE OUT: A BODY WORLDS Production at the International Centre for Life in Newcastle
I'm saying 'adolescent' because the growth plate has not fused (like a mouse), but is that true for a lion....?