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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