Localization and expression of ciliary neurotrophic factor (CNTF) in postmortem sciatic nerve from patients with motor neuron disease and diabetic neuropathy

Journal Article · Thu Aug 01 04:00:00 EDT 1996 · Journal of Neuropathology and Experimental Neurology

DOI:https://doi.org/10.1097/00005072-199608000-00007· OSTI ID:554905

Lee, D A ^[1]; Gross, L; Wittrock, D A; Windebank, A J ^[2]

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1. Univ. Medical Center, New Orleans, LA (United States)
2. Mayo Clinic, Rochester, MN (United States)

Ciliary neurotrophic factor (CNTF) is thought to play an important role in the maintenance of the mature motor system. The factor is found most abundantly in myelinating Schwann cells in the adult sciatic nerve. Lack of neuronal growth factors has been proposed as one possible etiology of amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). Growth factor replacement therapies are currently being evaluated as a treatment for motor neuron disease. In this report we determined whether the expression of CNTF in sciatic nerve differed in patients with motor neuron disease compared to controls or patients with another form of axonopathy. We identified 8 patients (7 with ALS and 1 with SMA) with motor neuron disease and 6 patients with diabetic motor neuropathy who had autopsy material available. Immunoperoxidase staining showed reduced CNTF expression in nerves of patients with motor neuron disease but not in patients with diabetic motor neuropathy. Decreased CNTF appears be associated with primary motor neuron disease rather than a generalized process of axon loss. This result supports suggestions that CNTF deficiency may be an important factor in the development of motor neuron disease. 20 refs., 4 figs., 1 tab.

• Sponsoring Organization: USDOE
• OSTI ID: 554905
• Journal Information: Journal of Neuropathology and Experimental Neurology, Journal Name: Journal of Neuropathology and Experimental Neurology Journal Issue: 8 Vol. 55; ISSN JNENAD; ISSN 0022-3069
• Country of Publication: United States
• Language: English

Related Subjects

55 BIOLOGY AND MEDICINE
BASIC STUDIES
DIABETES MELLITUS
ETIOLOGY
GENE RECOMBINATION
GENE REGULATION
GENETIC MAPPING
GROWTH FACTORS
HEREDITARY DISEASES
HISTOLOGICAL TECHNIQUES
IMMUNOASSAY
NERVE CELLS
NERVOUS SYSTEM DISEASES
PATIENTS
SAMPLE PREPARATION
SCIATIC NERVE
THERAPY