Differences in both inositol 1,4,5-trisphosphate mass and inositol 1,4,5-trisphosphate receptors between normal and dystrophic skeletal muscle cell lines.

Abstract:

:Human normal (RCMH) and Duchenne muscular dystrophy (RCDMD) cell lines, as well as newly developed normal and dystrophic murine cell lines, were used for the study of both changes in inositol 1,4,5-trisphosphate (IP3) mass and IP3 binding to receptors. Basal levels of IP3 were increased two- to threefold in dystrophic human and murine cell lines compared to normal cell lines. Potassium depolarization induced a time-dependent IP3 rise in normal human cells and cells of the myogenic mouse cell line (129CB3), which returned to their basal levels after 60 s. However, in the human dystrophic cell line (RCDMD), IP3 levels remained high up to 200 s after potassium depolarization. Expression of IP3 receptors was studied measuring specific binding of 3H-IP3 in the murine cell lines (normal 129CB3 and dystrophic mdx XLT 4-2). All the cell lines bind 3H-IP3 with relatively high affinity (Kd: between 40 and 100 nmol/L). IP3 receptors are concentrated in the nuclear fraction, and their density is significantly higher in dystrophic cells compared to normal. These findings together with high basal levels of IP3 mass suggest a possible role for this system in the deficiency of intracellular calcium regulation in Duchenne muscular dystrophy.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

Liberona JL,Powell JA,Shenoi S,Petherbridge L,Caviedes R,Jaimovich E

doi

10.1002/(sici)1097-4598(199807)21:7<902::aid-mus8>

subject

Has Abstract

pub_date

1998-07-01 00:00:00

pages

902-9

issue

7

eissn

0148-639X

issn

1097-4598

pii

10.1002/(SICI)1097-4598(199807)21:7<902::AID-MUS8>

journal_volume

21

pub_type

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