Specific absence of the α1 subunit of the dihydropyridine receptor in mice with muscular dysgenesis

C Michael Knudson; Nirupa Chaudhari; Alan H Sharp; Jeanne A Powell; Kurt G Beam; Kevin P Campbell

doi:10.1016/S0021-9258(18)94191-8

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Specific absence of the α1 subunit of the dihydropyridine receptor in mice with muscular dysgenesis

Letter/Communication

Open access

Specific absence of the α1 subunit of the dihydropyridine receptor in mice with muscular dysgenesis

C Michael Knudson, Nirupa Chaudhari, Alan H Sharp, Jeanne A Powell, Kurt G Beam and Kevin P Campbell

The Journal of biological chemistry, Vol.264(3), pp.1345-1348

01/25/1989

DOI: 10.1016/S0021-9258(18)94191-8

PMID: 2536362

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Abstract

Muscular dysgenesis is a lethal mutation in mice that results in a complete absence of skeletal muscle contraction due to the failure of depolarization of the transverse tubular membrane to trigger calcium release from the sarcoplasmic reticulum. In order to determine whether the defect in muscular dysgenesis leads to a specific loss of one of the components of excitation-contraction coupling or to a generalized loss of all components of excitation-contraction coupling, we have analyzed skeletal muscle from control and dysgenic mice for the sarcoplasmic reticulum and transverse tubular proteins which are believe to function in excitation-contraction coupling. We report that the proteins involved in sarcoplasmic reticulum calcium transport, storage, and release [Ca2+ + Mg2+)-ATPase, calsequestrin, and calcium release channel) are present in dysgenic muscle. Also present in dysgenic muscle is the 175/150-kDa glycoprotein subunit (alpha 2) of the dihydropyridine receptor. However, the 170-kDa dihydropyridine binding subunit (alpha 1) of the dihydropyridine receptor is absent in dysgenic muscle. These results suggest that the specific absence of the alpha 1 subunit of the dihydropyridine receptor is responsible for the defects in muscular dysgenesis and that the alpha 1 subunit of the dihydropyridine receptor is essential for excitation-contraction coupling in skeletal muscle.

Mutation

Molecular Weight

Calcium Channels - metabolism

Calcium - metabolism

Ca(2+) Mg(2+)-ATPase - metabolism

Muscles - abnormalities

Animals

Muscle Contraction

Mice, Mutant Strains

Receptors, Nicotinic - analysis

Calcium-Transporting ATPases - metabolism

Mice

Sarcoplasmic Reticulum - metabolism

Calsequestrin - metabolism

Receptors, Nicotinic - genetics

Details

Title: Subtitle: Specific absence of the α1 subunit of the dihydropyridine receptor in mice with muscular dysgenesis
Creators: C Michael Knudson - Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City 52242
Nirupa Chaudhari
Alan H Sharp
Jeanne A Powell
Kurt G Beam
Kevin P Campbell
Resource Type: Letter/Communication
Publication Details: The Journal of biological chemistry, Vol.264(3), pp.1345-1348
DOI: 10.1016/S0021-9258(18)94191-8
PMID: 2536362
NLM abbreviation: J Biol Chem
ISSN: 0021-9258
eISSN: 1083-351X
Publisher: United States
Grant note: HL-14388 / NHLBI NIH HHS HL-37187 / NHLBI NIH HHS NS-24444 / NINDS NIH HHS
Language: English
Date published: 01/25/1989
Academic Unit: Neurology; Molecular Physiology and Biophysics; Pathology; Iowa Neuroscience Institute; Radiation Oncology
Record Identifier: 9984047980702771

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