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Fast axonal transport in amyotrophic lateral sclerosis

An intra-axonal organelle traffic analysis

Departments of Neurology (Drs. Breuer and Wilbourn), Brain and Vascular Research (Drs. Breuer and Lynn, and MI. Atkinson), Pathology, Section of Neuropathology (Dr. Chou), Orthopaedic Surgery (Drs. Marks and Culver), and Plastic Surgery (Dr. Fleegler), The Cleveland Clinic Foundation, Cleveland, OH.

Fast transport of intra-axonal organelles was studied in motor nerve from amyotrophic lateral sclerosis (ALS) patients. Organelle traffic in ALS nerves demonstrated a significant increase in anterograde mean speed, while retrograde mean speed was decreased compared with that of controls. Retrograde traffic density (organelles per unit time) was also significantly decreased in the ALS specimens. Anterograde transport machinery is therefore intact and may be responding to the increased physiologic demand of larger motor units. Diminished retrograde speed and organelle traffic density are consistent with a defect in retrograde transport and could impair communication between axon terminals and perikarya.

Address correspondence and reprint requests to Dr. Breuer, Department of Neurology, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44106.

Supported by grants to A.C. Breuer from the NIH (NS 20384), Whitaker Foundation, ALS Society of America, and the Kingsbury, Smith, Lubrizol, and Palm Beach County Community Funds, and by private gifts and donations.

Received January 13, 1986. Accepted for publication in final form September 5, 1986.

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