Localized expression of specific P2X receptors in dystrophin-deficient DMD and mdx muscle

Taiwen Jiang; Davy Yeung; Chun Fu Lien; Dariusz C Górecki

doi:10.1016/j.nmd.2004.11.008

Localized expression of specific P2X receptors in dystrophin-deficient DMD and mdx muscle

Neuromuscul Disord. 2005 Mar;15(3):225-36. doi: 10.1016/j.nmd.2004.11.008.

Authors

Taiwen Jiang¹, Davy Yeung, Chun Fu Lien, Dariusz C Górecki

Affiliation

¹ Molecular Medicine Group, Institute of Biomedical and Biomolecular Sciences, School of Pharmacy and Biomedical Sciences, St Michael's Building, White Swan Road, Portsmouth PO1 2DT, UK.

PMID: 15725584
DOI: 10.1016/j.nmd.2004.11.008

Abstract

Using a combination of molecular and immunohistochemical methods, we have obtained evidence for a distinctive change in the expression patterns of ATP-gated (P2X) receptor subunits in dystrophic muscle from both Duchenne muscular dystrophy (DMD) patients and the mdx mouse model. In control myofibres there was no staining for any P2X subtype studied here, although P2X1 stained the smooth muscle of the blood vessels and P2X6 nerves and the tunica intima in small arteries. In contrast, P2X1 and P2X6 were co-expressed strongly in small regenerating muscle fibres in the dystrophic muscles, whereas this expression decreased in fully regenerated fibres. Moreover, immunoreactivity for the P2X2 receptor re-appeared in dystrophic muscle, where it co-localised with the Type 1 fibres. There is, thus, a burst of production of several P2X receptor subtypes in regenerating dystrophic muscle, which may have implications for drug targets for this muscle pathology.

Publication types

Comparative Study
Research Support, Non-U.S. Gov't

MeSH terms

Animals
Blotting, Northern / methods
Blotting, Western / methods
Dystrophin / deficiency*
Embryo, Mammalian
Gene Expression Regulation
Humans
Immunohistochemistry / methods
Male
Methyl Green / metabolism
Mice
Mice, Inbred C57BL
Mice, Inbred mdx
Muscle, Skeletal / metabolism*
Muscular Dystrophy, Animal / genetics
Muscular Dystrophy, Animal / metabolism*
Muscular Dystrophy, Duchenne / genetics
Muscular Dystrophy, Duchenne / metabolism*
RNA, Messenger / biosynthesis
Rats
Receptors, Purinergic P2 / genetics
Receptors, Purinergic P2 / metabolism*
Receptors, Purinergic P2X2
Reverse Transcriptase Polymerase Chain Reaction / methods
Sequence Alignment / methods
Sequence Analysis, Protein / methods
Succinate Dehydrogenase / metabolism
Vasoactive Intestinal Peptide / metabolism

Substances

Dystrophin
P2RX2 protein, human
P2rx2 protein, mouse
RNA, Messenger
Receptors, Purinergic P2
Receptors, Purinergic P2X2
purinoceptor P2X6
Vasoactive Intestinal Peptide
Methyl Green
Succinate Dehydrogenase