Cell and fiber type distribution of dystrophin

doi:10.1016/0896-6273(88)90191-2

Neuron

Volume 1, Issue 5, July 1988, Pages 411-420

https://doi.org/10.1016/0896-6273(88)90191-2 Get rights and content

Abstract

Duchenne muscular dystrophy is the result of dystrophin deficiency. We have determined the cell types likely to express the pathogenic effects of this neuromuscular disease by determining the pattern of dystrophin expression in normal cells. We find that all physiological types of muscle cells express dystrophin at similar levels, and that the dystrophin content of various tissues correlates with the myogenic cell population of each tissue. The dystrophin content of brain and spinal cord, however, is found not to correlate with any type of muscle cell, and it is suggested that neurons express dystrophin. The potential involvement of striated muscle fibers, the vasculature, and the nervous system in the etiology of Duchenne muscular dystrophy makes it likely that the disease is a complex disorder of combined pathogenesis. We also find that the dystrophic chicken does not represent an animal model for dystrophin deficiency.

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Neuron

Cell and fiber type distribution of dystrophin

Abstract

J. Neurol. Sci.

J. Neurol. Sci.

Brain Res.

Lancet

Cell

Exp. Neurol.

Am. Heart J.

Dev. Biol.

Cell

Cell

Lancet

Gastroenterology

Int. J. Dev. Neurosci.

Cell

Inherited muscle abnormality in the domestic fowl

J. Hered.

Changes in the musculature of the gastrointestinal tract and in the myocardium in progressive muscular dystrophy

Arch. Pathol.

Duchenne muscular dystrophy: deficiency of dystrophin at the muscle cell surface

Cell

Failure to confirm a vascular cause of muscular dystrophy

Arch. Neurol.

Clinical investigation in Duchenne dystrophy: 2. Determination of the “power” of therapeutic trials based on the natural history

Muscle Nerve

X chromosome-linked muscular dystrophy (mdx) in the mouse

A cDNA clone from the Duchenne/Becker muscular dystrophy gene

Nature

Morphology and physiology of skeletal muscle in aging rodents

Muscle Nerve

Protein components and their roles in sarcoplasmic reticulum function

Expression of the murine Duchenne muscular dystrophy gene in muscle and brain

Science

Animal models of muscle disease, part I (avian dystrophy)

Muscle Nerve

Prednisone treatment in Duchenne muscular dystrophy: long term benefit

Arch. Neurol.

Contractile responses to epinephrine, serotonin, and potassium in arterial smooth muscle

Mental retardation in Duchenne muscular dystrophy

Muscle Biopsy: A Practical Approach

Muscle Biopsy: A Modern Approach

Quantitative ultrastructure of mammalian skeletal muscle

Duchenne muscular dystrophy

Integrative histochemical approach to the defect of Duchenne muscular dystrophy

Focal lesions of muscle in peripheral vascular disease

J. Neurol.