Table II Representative spectral count data for select lysosomal proteins

Bold values indicate proteins encoded by mutant genes in the respective case. Counts are normalized by multiplying by the average number of spectra assigned in all samples divided by the total number of spectra assigned in that particular sample. Bold indicates verified defects. HEXA, β-hexosaminidase α.

IdentifierSelect lysosomal proteins
CTSDASAH1PPT1HEXATPP1SGSHCLN5
HSB 2381054.0231.0246.883.351.69.512.7
HSB 298898.6174.3143.5117.025.629.912.8
HSB 3041207.5191.9263.285.744.015.96.8
HSB 3311190.2191.4211.986.339.314.48.3
HSB 7121049.6196.6170.876.336.321.013.4
HSB 863765.9312.5229.382.279.223.67.2
CABM-BR1903.8440.9169.3103.374.648.814.3
CABM-BR11537.3350.6399.793.4109.880.39.0
CABM-BR19969.5271.0228.998.841.130.30.0
CABM-BR21303.3563.7145.7102.71.260.921.5
CABM-BR23744.4160.2200.6140.0127.959.25.4
CABM-BR24348.9157.6150.4185.330.199.926.5
CABM-BR25418.9127.2148.6107.00.082.216.9
CABM-BR32734.2441.1391.182.6286.444.25.8
CABM-BR33778.0322.3309.3107.454.341.320.1
CABM-BR34636.1473.1203.053.090.022.042.0
CABM-BR36617.3376.1148.8104.4133.113.911.1
CABM-BR8922.3300.4398.774.460.633.121.1
HSB 1183673.5480.8220.0114.5140.611.318.1
HSB 120719.5485.4286.581.3249.124.67.5
HSB 1481058.7134.8291.40.0203.68.79.6
HSB 2356771.2280.4419.090.767.733.415.6
HSB 2548527.9322.9325.482.0235.938.520.1
HSB 4165898.2238.0369.387.187.10.020.9
HSB 43911388.2306.7105.6102.90.932.424.1
UMB 1270333.2268.6212.9124.392.535.822.9
UMB 338872.2385.3265.0131.486.116.616.6
UMB 506840.7441.4147.5110.443.118.119.1
UMB 5631074.0262.5545.9107.1117.60.017.8
UMB 575901.3393.5151.2130.394.717.217.2
UMB 613203.0118.256.6168.110.084.915.0
UMB 784702.6191.7265.8141.610.940.317.4
UMB 821872.9405.1217.3130.986.319.923.7