268
CASE FILES: BIOCHEMISTRY
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iduronate sulfatase
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Hunter syndrome (MPS II)
Hurler, Scheie,
Hurler-Scheie syndromes (MPS I)
Sanfilippo syndrome, Type A (MPS III A)
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heparan N-sulfatase
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Sanfilippo syndrome, Type C (MPS III C)
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Sanfilippo syndrome, Type B (MPS III B)
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Sanfilippo syndrome, Type D (MPS III D)
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Figure 29-1. Lysosomal degradation of heparan sulfate. Dark arrows indicate the
site of action of the enzyme, which is listed in italics. Mucopolysaccharidoses
(MPS) due to a deficiency of the indicated enzymes are listed in bold to the right
of the enzyme.
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