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Hunter and Hurler syndrome

Biochemistry
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Feature

Hunter Syndrome

(MPS II)

Hurler Syndrome

(MPS I)

Inheritance

X-linked recessive

(affects males)

Autosomal recessive

Deficient

Enzyme

Iduronate-2-sulfatase

α-L-Iduronidase

Accumulated

Substances

- Dermatan sulfate

- Heparan sulfate

- Dermatan sulfate

- Heparan sulfate

Ophthalmic

Findings

No corneal clouding

Corneal clouding present