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Table 1 Lysosomal proteins in human brain and CSF

From: Proteomics analysis of a human brain sample from a mucolipidosis type IV patient reveals pathophysiological pathways

Protein

CB

CRB

CSF

Ratio (MLIV/Con)

Lysosomal hydrolases

HEXA

1.77

2.93

0.90

HEXB

5.96

3.95

6.96

NPC1

−

3.93

n.d

NPC2

2.58

4.34

2.59

GNS

2.94

3.01

 + 

GM2A

3.46

9.24

2.66

GLB1

3.98

2.05

n.d

GAA

4.56

2.74

4.90

GUSB

7.07

 + 

n.d

ASAH1

6.41

5.11

2.61

PLBD2

0.45

2.51

n.d

TPP1

2.15

2.18

0.70

ARSA

3.56

2.29

1.70

LIPA

1.39

4.49

n.d

GALNS

−

−

n.d

Cathepsins

CTSA

 + 

1.49

 + 

CTSB

2.07

1.67

1.23

CTSD

1.21

1.46

1.30

CTSH

3.08

1.84

2.11

CTSL

0.40

2.73

1.09

CTSZ

0.81

2.27

3.17

Lysosomal proteins

LAMP1

2.82

2.44

 

LAMP2

3.31

4.71

1.53

PRCP

7.52

11.7

2.71

PSAP

3.37

3.92

7.30

CAV1

2.99

1.09

0.07

ACP2

0.88

3.19

n.d

SCARB2

2.82

2.13

n.d

DNASE2

1.09

 + 

n.d

NHLRC3

 + 

 + 

n.d

PPT1

1.88

2.58

1.39

FTH1

1.27

1.86

4.65

FTL

0.92

1.96

2.80

LMBRD1

−

n.d

n.d

  1. A total of 42 lysosomal proteins [73] were detected. The proteins listed in the Table are those that differ between the control and MLIV in one or other tissue. When a ratio could not be calculated (i.e., a value of zero was obtained in either the control or in MLIV), changes are indicated by + for a protein that was detected only in the MLIV sample, and by—when the protein was detected only in the control sample; n.d., not detected in either sample. Con, control; CB, cerebellum; CRB, cerebrum