Authors: Pagliardini, Silvia; Giavazzi, Alessio; Setola, Veronica; Lizier, Carlotta; Di Luca, Monica¹; DeBiasi, Silvia²; Battaglia, Giorgio

Source: Human Molecular Genetics, Volume 9, Number 1, 1 January 2000 , pp. 47-56(10)

Publisher: Oxford University Press

Abstract:

The subcellular localization of the survival motor neuron (SMN) protein, encoded by the spinal muscular atrophy determining gene, was investigated in motor neurons of the developing and adult rat spinal cord by light and electron microscopy immunocytochemistry. The experiments were carried out with a panel of anti-SMN antibodies, all recognizing an SMN-specific protein band at 39 kDa in HeLa cells and rat spinal cord protein extracts. SMN protein expression decreased during postnatal spinal cord development, but it remained unchanged in distribution and intensity in motor neurons at all ages examined. SMN protein was mainly organized in immunoreactive aggregates sparse in the nucleoplasm and cytoplasm of both mature and developing motor neurons, and it was more rarely localized within the endoplasmic reticulum and in apposition to the external mitochondrial membrane. Most strikingly, the SMN protein was found in association with cytoskeletal elements in spinal dendrites and axons, where it was particularly evident during postnatal development. The present findings suggest that SMN protein may be transported via axoplasmic flow in maturing neurons. Given the RNA-binding activity of SMN, the SMN protein could be involved in the transport of specific mRNAs in axons and dendrites of motor neurons. The reduced transport of specific mRNAs within motor neurons during development could play a role in the motoneuronal degeneration and impaired axonal sprouting observed in spinal muscular atrophy.

Document Type: Research article

Affiliations: 1: Institute of Pharmacological Sciences, University of Milano, via Balzaretti 9, 20133 Milano, Italy and 2: Department of General Physiology and Biochemistry, University of Milano, via Celoria 26, 20133 Milano, Italy

Publication date: 2000-01-01

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• Human Molecular Genetics concentrates on full-length research papers covering a wide range of topics in all aspects of human molecular genetics.

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• By this author: Pagliardini, Silvia ;  Giavazzi, Alessio ;  Setola, Veronica ;  Lizier, Carlotta ;  Di Luca, Monica ;  DeBiasi, Silvia ;  Battaglia, Giorgio

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