Fig. 6. Ultrastructural analysis of neuromuscular junctions (NMJ), myotendinous
junctions (MTJ) and glomerular filtration barriers. (A-D) A control
synapse (A) shows a Schwann cell (s) capping the vesicle-rich nerve terminal
(nt) adjacent to the muscle (m) endplate containing numerous junctional folds
(jf). In the Lamb2-/- synapse (B), junctional folds are
absent and the Schwann cell extends processes (arrow) between the nerve
terminal and the muscle. Synaptic deposition of laminin ß2 in
Lamb2-/-; MCK-B2 mice restores synaptic architecture to
normal (C). In Lamb2-/-; NEPH-B2 mice, glomerular
deposition of ß2 and prevention of proteinuria has no restorative effect
on the synapse (D). (E-G) MTJ from a control (E) exhibits numerous
infoldings of the muscle fiber (m) with continuous BMs. In the
Lamb2-/- MTJ (F), infoldings are less complex, and the BMs
(arrows) appear fuzzy. MCK-B2 transgene-derived ß2 restores much of the
normal MTJ architecture (G). (H-K) Glomerular capillary segment from a
control (H) shows the interdigitated podocyte foot processes (fp) adjacent to
the GBM. Effaced foot processes (efp) are evident in the
Lamb2-/- (I) and Lamb2-/-; MCK-B2 (J)
mice, which are proteinuric. Deposition of rat ß2 into the GBM in
Lamb2-/-; NEPH-B2 mice prevents proteinuria and foot
process effacement (K). Scale bars in D and K are 1 µm for A-D and H-K,
respectively; scale bar in G is 2 µm for E-G.