EM EM EM EM

Laboratory for Ultrastructural Neuropathology (LUN)

2. Expertise and technical facilities:

The longstanding expertise of the LUN is commonly solicited for diagnostical as well as for research purposes.

Histopathological expertise:

1. Ultrastructure of brain, peripheral nerves, skeletal muscles, skin and conjunctiva in:
- Lysosomal storage diseases, peroxisomal disorders (adrenoleukomyeloneuropathies) and non-lysosomal storage disorders.
- Neurodegenerative dementing disorders.
- An inherited cerebrovascular disease: CADASIL.
- Hereditary - and acquired peripheral neuropathies: Charcot-Marie-Tooth disease and amyloidoses.
- Neuromuscular disorders: muscular dystrophies, congenital myopathies, metabolic myopathies (mitochondrial muscular diseases and glycogenoses) and inflammatory muscular disease (polymyositis, dermatomyositis, “inclusion body myositis”).

2. Morphological criteria and requirements to characterize normal-, artifactual- and pathological aspects.

3. Differential diagnosis in lysosomal storage disease, demyelinating- and axonal neuropathies and congenital myopathies.

4. Prenatal investigation of chorionic villus and amnion cells in neuronal ceroid lipofuscinoses.

Practical procedural expertise

1. Conventional techniques for transmission EM (fixation, resin embedding, ultramicrotomy, staining procedures).

2. Specific ultrastructural stainings such as Thiéry’s method to demonstrate polysaccharides at the ultrastructural level.

3. Immuno-EM (PLT method, freeze substitution, cryo-ultramicrotomy).

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