Thymic changes in muscular dystrophy and evidence for an abnormality related to prostaglandin synthesis or action.

Abstract:

:In Bar Harbor 129 dystrophic mice, thymic development is abnormal. Before weaning, the thymus is slightly smaller than in phenotypically normal littermates; after weaning, however, the lymphoid elements undergo rapid atrophy. The epithelial elements, in contrast, display hyperlasia. Thymectomy has no influence on the course of the disease, and it is possible that the thymic changes are a reflection of a fundamental metabolic abnormality. Thymic lymphoid tissue development seems to require normal levels of PGE1. Levels that are either too high or too low both result in abnormalities. We have investigated the effects of PGE1 in smooth muscle and have demonstrated that while some PGE1 is required for both calcium release and calcium removal, high levels of PGE1 block both processes. We propose that the muscular dystrophies are related to defects in PG synthesis and action. Myotonic dystrophy may be due to PGE11 excess, whereas Duchenne dystrophy may in part be due to PGE1 deficiency.

journal_name

Ann N Y Acad Sci

authors

Horrobin DF,Morgan RO,Karmali RA,Ally AI,Manku MS,Karmazyn M,Cunnane SC

doi

10.1111/j.1749-6632.1979.tb56574.x

subject

Has Abstract

pub_date

1979-01-01 00:00:00

pages

534-49

eissn

0077-8923

issn

1749-6632

journal_volume

317

pub_type

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