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Table 2 Serum lysosomal enzyme activities in ML II affected kittens and clinical healthy littermates with normal ranges

From: A GNPTAB nonsense variant is associated with feline mucolipidosis II (I-cell disease)

Enzyme Animal ID # and clinical status Normal controla
Median (IQR), n = 10
Affected Healthy
7160 7162 7163 Median (IQR) 7161 7164 Median (IQR)  
Activityb
α-L-iduronidase 95.5 95.0 65.7 95.0 (29.8)c 14.8 13.7 14.3 (1.1) 14.0 (4.3)
arylsulfatase B 209.8 170.4 185.4 185.4 (39.4)c 10.9 9.9 10.4 (1.0) 9.8 (5.8)
β-glucuronidase 6126 7196 6061 6126 (1185)c 235.4 263.1 249.3 (27.7) 255.4 (29.3)
α-D-mannosidase 61,921 62,017 50,822 61,921 (11195)c 7079 6217 6648 (862) 6114 (3635)
α-D-fucosidase 2543 3015 2459 2543 (556)c 140.6 166.8 153.7 (26.2) 156.5 (44.2)
N-acetyl-β-D-glucosaminidase 9294 8380 6923 8380 (2371)c 1155 1015 1085 (140) 1138 (200)
  1. aReference values were based on 10 clinically healthy kittens, and presented as median with interquartile range (IQR)
  2. bEnzyme activities were presented as nMol 4-MU/mL serum/hour
  3. cp < 0.01 when compared to unrelated control cats and also to the combined group of unrelated control cats and clinically healthy littermates