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On aligned scaffolds of large fibers (∼740 nm) cell directionality closely follows the underlying fibers, irrespective of the alignment method.
Sensory tests in face for small and large fibers were within normal limits in all patients (no distal loss of pinprick or thermal sensation, nor punctate hyperalgesia or brush evoked allodynia were observed).
In GarsC201R/+ mice, there is a greater proportion of large fibers present, and some of the fibers have a larger CSA observed in wildtype muscles.
However, in SOD1G93A GarsC201R/+ mice, the TA muscles have a greater proportion of large fibers than observed in SOD1G93A mice, suggesting that the presence of the GarsC201R/+ mutation has prevented the reduction in muscle fiber area that otherwise occurs in TA muscles of SOD1G93A mice by 120 days.
Nerve conduction studies, however, only assess large fibers.
Often there is a mixed presentation with abnormalities of both small and large fibers.
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