Skip to main content

Table 1 Open Muscle biopsies subjected to pathological analyses, and generation of muscle satellite cells for in vitro studies

From: Impaired signaling for neuromuscular synaptic maintenance is a feature of Motor Neuron Disease

Donor IDs Sex Age Diagnosis Site of onset ALSFRS-R (at biopsy) Interval—Symptom Onset to Biopsy months No NMJs imaged Muscle pathology
Con-3# M 44      19$ Normal
Con-4 M 54      22$, 7* Inclusion Body Myositis
Con-5 M 39      12$ Focal Denervation
MND-1^ M 57 Sporadic MND Spinal 34 10 4* Chronic progressive denervation, early fiber type grouping
MND-3^ F 56 Sporadic MND Spinal N/A 10 40$, 5* Chronic progressive denervation, early fiber type grouping
MND-4 M 61 Sporadic MND Spinal 41 60 24$ Chronic denervation; collateral reinnervation, fiber type grouping
MND-5 M 63 Sporadic MND Spinal 40 31 2* Chronic progressive denervation, fiber type grouping
MND-6 F 77 Sporadic MND Bulbar 29 31 3* Chronic progressive denervation, fiber type grouping
MND-7^ F 68 Sporadic MND Spinal 40 12   Denervation atrophy, early fiber type grouping
  1. Con = Control (non-MND other disease control); ALSFRS-R—ALS Functional Rating Scale Revised; #Deltoid muscle biopsy, the reminder are biopsies from Vastus Lateralis muscle; $NMJs found in whole mount intact muscle fiber samples; *NMJs found in cryosections used in locating MuSK at the NMJ; ^Muscle also used to derive muscle satellite cells for in vitro studies