Congenital canine myasthenia gravis: II. Acetylcholine receptor metabolism

K. Oda, Vanda A Lennon, E. H. Lambert, A. C. Palmer

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Acetylcholine receptor (AChR) metabolism was studied in muscle from juvenile and adult dogs with congenital myasthenia gravis (CMG) and their unaffected littermates. Although the amount of AChR in the junctional region of innervated CMG muscle fibers was 25% of normal, or less, denervation of CMG fibers resulted in the appearance of AChR in extrajunctional membranes at as high a concentration as in denervated normal fibers. The rate of degradation of junctional AChR in CMG fibers explanted to organ culture did not differ significantly from normal. In monolayer cultures derived from enzyme-dissociated CMG muscle, myotubes of normal morphology developed, and the synthesis and degradation of AChR did not differ from normal. Addition of sera from dogs with the acquired autoimmune form of MG accelerated the degradation of AChR on cultured myotubes, but CMG dog sera were without effect. These data suggest that the low junctional membrane density of AChR in CMG does not reflect a primary inability of muscle to synthesize AChR, nor an accelerated degradation of AChR in the postsynaptic membrane, but rather a low insertion rate of AChR in the postsynaptic membrane.

Original languageEnglish (US)
Pages (from-to)717-724
Number of pages8
JournalMuscle and Nerve
Volume7
Issue number9
StatePublished - 1984

Fingerprint

Congenital Myasthenic Syndromes
Cholinergic Receptors
Canidae
Muscles
Membranes
Skeletal Muscle Fibers
Dogs
Organ Culture Techniques
Denervation
Serum

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Congenital canine myasthenia gravis : II. Acetylcholine receptor metabolism. / Oda, K.; Lennon, Vanda A; Lambert, E. H.; Palmer, A. C.

In: Muscle and Nerve, Vol. 7, No. 9, 1984, p. 717-724.

Research output: Contribution to journalArticle

Oda, K, Lennon, VA, Lambert, EH & Palmer, AC 1984, 'Congenital canine myasthenia gravis: II. Acetylcholine receptor metabolism', Muscle and Nerve, vol. 7, no. 9, pp. 717-724.
Oda, K. ; Lennon, Vanda A ; Lambert, E. H. ; Palmer, A. C. / Congenital canine myasthenia gravis : II. Acetylcholine receptor metabolism. In: Muscle and Nerve. 1984 ; Vol. 7, No. 9. pp. 717-724.
@article{cb6bcf4463ea4d2cb3bbd90bd9676713,
title = "Congenital canine myasthenia gravis: II. Acetylcholine receptor metabolism",
abstract = "Acetylcholine receptor (AChR) metabolism was studied in muscle from juvenile and adult dogs with congenital myasthenia gravis (CMG) and their unaffected littermates. Although the amount of AChR in the junctional region of innervated CMG muscle fibers was 25{\%} of normal, or less, denervation of CMG fibers resulted in the appearance of AChR in extrajunctional membranes at as high a concentration as in denervated normal fibers. The rate of degradation of junctional AChR in CMG fibers explanted to organ culture did not differ significantly from normal. In monolayer cultures derived from enzyme-dissociated CMG muscle, myotubes of normal morphology developed, and the synthesis and degradation of AChR did not differ from normal. Addition of sera from dogs with the acquired autoimmune form of MG accelerated the degradation of AChR on cultured myotubes, but CMG dog sera were without effect. These data suggest that the low junctional membrane density of AChR in CMG does not reflect a primary inability of muscle to synthesize AChR, nor an accelerated degradation of AChR in the postsynaptic membrane, but rather a low insertion rate of AChR in the postsynaptic membrane.",
author = "K. Oda and Lennon, {Vanda A} and Lambert, {E. H.} and Palmer, {A. C.}",
year = "1984",
language = "English (US)",
volume = "7",
pages = "717--724",
journal = "Muscle and Nerve",
issn = "0148-639X",
publisher = "John Wiley and Sons Inc.",
number = "9",

}

TY - JOUR

T1 - Congenital canine myasthenia gravis

T2 - II. Acetylcholine receptor metabolism

AU - Oda, K.

AU - Lennon, Vanda A

AU - Lambert, E. H.

AU - Palmer, A. C.

PY - 1984

Y1 - 1984

N2 - Acetylcholine receptor (AChR) metabolism was studied in muscle from juvenile and adult dogs with congenital myasthenia gravis (CMG) and their unaffected littermates. Although the amount of AChR in the junctional region of innervated CMG muscle fibers was 25% of normal, or less, denervation of CMG fibers resulted in the appearance of AChR in extrajunctional membranes at as high a concentration as in denervated normal fibers. The rate of degradation of junctional AChR in CMG fibers explanted to organ culture did not differ significantly from normal. In monolayer cultures derived from enzyme-dissociated CMG muscle, myotubes of normal morphology developed, and the synthesis and degradation of AChR did not differ from normal. Addition of sera from dogs with the acquired autoimmune form of MG accelerated the degradation of AChR on cultured myotubes, but CMG dog sera were without effect. These data suggest that the low junctional membrane density of AChR in CMG does not reflect a primary inability of muscle to synthesize AChR, nor an accelerated degradation of AChR in the postsynaptic membrane, but rather a low insertion rate of AChR in the postsynaptic membrane.

AB - Acetylcholine receptor (AChR) metabolism was studied in muscle from juvenile and adult dogs with congenital myasthenia gravis (CMG) and their unaffected littermates. Although the amount of AChR in the junctional region of innervated CMG muscle fibers was 25% of normal, or less, denervation of CMG fibers resulted in the appearance of AChR in extrajunctional membranes at as high a concentration as in denervated normal fibers. The rate of degradation of junctional AChR in CMG fibers explanted to organ culture did not differ significantly from normal. In monolayer cultures derived from enzyme-dissociated CMG muscle, myotubes of normal morphology developed, and the synthesis and degradation of AChR did not differ from normal. Addition of sera from dogs with the acquired autoimmune form of MG accelerated the degradation of AChR on cultured myotubes, but CMG dog sera were without effect. These data suggest that the low junctional membrane density of AChR in CMG does not reflect a primary inability of muscle to synthesize AChR, nor an accelerated degradation of AChR in the postsynaptic membrane, but rather a low insertion rate of AChR in the postsynaptic membrane.

UR - http://www.scopus.com/inward/record.url?scp=0021739460&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0021739460&partnerID=8YFLogxK

M3 - Article

C2 - 6543920

AN - SCOPUS:0021739460

VL - 7

SP - 717

EP - 724

JO - Muscle and Nerve

JF - Muscle and Nerve

SN - 0148-639X

IS - 9

ER -