TY - JOUR
T1 - Fibrillin-2 is dispensable for peripheral nerve development, myelination and regeneration
AU - Chernousov, Michael A.
AU - Baylor, Kelly
AU - Stahl, Richard C.
AU - Stecker, Mark M.
AU - Sakai, Lynn Y.
AU - Lee-Arteaga, Sui
AU - Ramirez, Francesco
AU - Carey, David J.
N1 - Funding Information:
We are indebted to Cindy Rhone (Weis Center for Research) for help with laboratory animals. We also thank John Shaw (Weis Center for Research) for assistance with electron microscopy, Noe Charbonneau (Oregon Health and Science University ) for help with immunofluorescence and Tanya Orleanskaya (Geisinger Health Sciences Libraries) for editorial assistance. This work was supported by NIH grant AR42044 to F.R.
PY - 2010/6
Y1 - 2010/6
N2 - The extracellular matrix of peripheral nerve is formed from a diverse set of macromolecules, including glycoproteins, collagens and proteoglycans. Recent studies using knockout animal models have demonstrated that individual components of the extracellular matrix play a vital role in peripheral nerve development and regeneration. In this study we identified fibrillin-1 and fibrillin-2, large modular structural glycoproteins, as components of the extracellular matrix of peripheral nerve. Previously it was found that fibrillin-2 null mice display joint contractures, suggesting a possible defect of the peripheral nervous system in these animals. Close examination of the peripheral nerves of fibrillin-2 deficient animals described here revealed some structural abnormalities in the perineurium, while general structure of the nerve and molecular composition of nerve extracellular matrix remained unchanged. We also found that in spite of the obvious motor function impairment, fibrillin-2 null mice failed to display changes of nerve conduction properties or nerve regeneration capacity. Based on the data obtained we can conclude that peripheral neuropathy should be excluded as the cause of the impairment of locomotory function and joint contractures observed in fibrillin-2 deficient animals.
AB - The extracellular matrix of peripheral nerve is formed from a diverse set of macromolecules, including glycoproteins, collagens and proteoglycans. Recent studies using knockout animal models have demonstrated that individual components of the extracellular matrix play a vital role in peripheral nerve development and regeneration. In this study we identified fibrillin-1 and fibrillin-2, large modular structural glycoproteins, as components of the extracellular matrix of peripheral nerve. Previously it was found that fibrillin-2 null mice display joint contractures, suggesting a possible defect of the peripheral nervous system in these animals. Close examination of the peripheral nerves of fibrillin-2 deficient animals described here revealed some structural abnormalities in the perineurium, while general structure of the nerve and molecular composition of nerve extracellular matrix remained unchanged. We also found that in spite of the obvious motor function impairment, fibrillin-2 null mice failed to display changes of nerve conduction properties or nerve regeneration capacity. Based on the data obtained we can conclude that peripheral neuropathy should be excluded as the cause of the impairment of locomotory function and joint contractures observed in fibrillin-2 deficient animals.
KW - Extracellular matrix
KW - Fibrillin
KW - Peripheral nerve
UR - http://www.scopus.com/inward/record.url?scp=77954459598&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77954459598&partnerID=8YFLogxK
U2 - 10.1016/j.matbio.2010.02.006
DO - 10.1016/j.matbio.2010.02.006
M3 - Article
C2 - 20188829
AN - SCOPUS:77954459598
SN - 0945-053X
VL - 29
SP - 357
EP - 368
JO - Collagen and Related Research
JF - Collagen and Related Research
IS - 5
ER -