Fibrillin-2 is dispensable for peripheral nerve development, myelination and regeneration.
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.
Chernousov
Michael A
MA
Weis Center for Research, Geisinger Clinic, 100 North Academy Avenue, Danville, PA 17822-2613, USA. mchernousov@geisinger.edu
Baylor
Kelly
K
Stahl
Richard C
RC
Stecker
Mark M
MM
Sakai
Lynn Y
LY
Lee-Arteaga
Sui
S
Ramirez
Francesco
F
Carey
David J
DJ
D000818:Animals; D000831:Animals, Newborn; D005109:Extracellular Matrix / Q000502:physiology*; D000071838:Fibrillin-1; D000071840:Fibrillin-2; D000071837:Fibrillins; D019085:Fluorescent Antibody Technique, Direct; D006614:Hindlimb / Q000502:physiology; D008124:Locomotion / Q000502:physiology; D051379:Mice; D018345:Mice, Knockout; D008840:Microfilament Proteins / Q000502:physiology*; D017348:Microscopy, Electron, Scanning Transmission; D018482:Muscle, Skeletal / Q000294:innervation* / Q000648:ultrastructure; D009186:Myelin Sheath / Q000502:physiology*; D009416:Nerve Regeneration / Q000502:physiology*; D009431:Neural Conduction / Q000502:physiology; D017933:Peripheral Nervous System / Q000502:physiology* / Q000648:ultrastructure; D012584:Sciatic Nerve; D054392:Adipokines
D000071838:Fibrillin-1; D000071840:Fibrillin-2; D000071837:Fibrillins; D008840:Microfilament Proteins; C000605724:Fbn1 protein, mouse; D054392:Adipokines; C000605728:Fbn2 protein, mouse