Q Cd Cq Cd

Continued

Table 1. Neurodegenerative diseases characterized by misfolding and deposition of aggregated protein—cont'd oo

Table 1. Neurodegenerative diseases characterized by misfolding and deposition of aggregated protein—cont'd

Disease

Mutated gene

Chromosome

Toxic protein, protein deposit

Risk factor

Morphology

Clinical features

Hereditary (familial) spastic paraparesis (FSP)

SPG 1-27, HSPG-6G, LICAM

Xq, 2, 3, 8, 9, 11, 12, 14, 15, 16, 19

Atlastin, spastin, SPG

SPG-L1 cell adhesion molecule; SPG-2 proteolipid protein, SPG-4 spastin, others unknown

Degeneration corticospinal + posterior spinal tracts

Progressive spastic paraparesis, pescavus, reduced vibration sense

Prion diseases

Creutzfeldt-Jakob disease (CJD)

- sporadic

- familial (GSS)

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