Muscular dystrophy with laminin deficiency decreases the content of butyrylcholinesterase tetramers in sciatic nerves of Lama2dy mice.

Abstract:

:The Lama2dy mouse, a model of congenital muscular dystrophy (CMD) by merosin deficiency (MCMD), shows muscle degeneration and dysmyelination of peripheral nerves. Although it has been reported that MCMD reduces acetylcholinesterase (AChE) activity of mouse sciatic nerve, no information is available regarding its action on butyrylcholinesterase (BuChE). Amphiphilic BuChE monomers (G(1)(A), 39%), dimers (G(2)(A), 18%), and tetramers (G(4)(A), 33%), along with hydrophilic tetramers (G(4)(H), 10%), were identified in mouse sciatic nerve. It also contained abundant G(4)(A) (75%) and less G(1)(A), G(2)(A), G(4)(H) and A(12) AChE components. In dystrophic nerves, the BuChE activity increased 2-fold but the proportion of the G(4)(A) form dropped from 33% to 10%. AChE activity decreased and the composition of enzyme forms was unaffected. Lectin interaction studies showed that, in contrast to skeletal muscle, the defect of merosin did not greatly alter the glycosylation of nerve cholinesterases. The anomalous synthesis of BuChE forms in dystrophic nerve may be related with peripheral neuropathy of MCMD.

journal_name

Neurosci Lett

journal_title

Neuroscience letters

authors

Moral-Naranjo MT,Cabezas-Herrera J,Vidal CJ,Campoy FJ

doi

10.1016/s0304-3940(02)00885-6

keywords:

subject

Has Abstract

pub_date

2002-10-18 00:00:00

pages

155-8

issue

3

eissn

0304-3940

issn

1872-7972

pii

S0304394002008856

journal_volume

331

pub_type

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