Table 1 Glycosphingolipid dysregulation in the cerebrospinal fluid of ALS patients.

From: Inhibition of β-Glucocerebrosidase Activity Preserves Motor Unit Integrity in a Mouse Model of Amyotrophic Lateral Sclerosis

Items

Neurological disorders

ALS

p-value

Cohort size

12

14

 

Female (%)

33.3

40

 

Male (%)

66.7

60

 

Age (years)

68.3 ± 1.4

70.3 ± 0.6

n.s.

ALSFRS-R

N/A

39.9 ± 0.3

—

Site of onset ( bulbar/spinal )

N/A

5/9

—

CSF glucose ( mmol/L )

3.8 ± 0

4.1 ± 0

n.s.

CSF protein ( g / L )

0.44 ± 0.01

0.51 ± 0.02

n.s.

GlcCer

84.8 ± 1

98.9 ± 1.1

*

GM1a

83.9 ± 1.1

104.3 ± 2.3

*

GD1a

74.6 ± 1.8

88.2 ± 2.3

n.s.

GD1b

71.7 ± 1.7

97 ± 2.3

n.s.

GT1b

54 ± 2.3

66.8 ± 3.7

n.s

  1. The characteristics and levels of glycosphingolipids of individuals diagnosed with non-ALS neurological disorders and ALS are shown. Glycosphingolipids are expressed as HPLC peak area/µl CSF, Mean ± SEM. *Corrected p-value < 0.05.
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