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Related Concept Videos

ATP Synthase: Mechanism01:48

ATP Synthase: Mechanism

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In animals, the mitochondrial F1F0 ATP synthase is the key protein that synthesizes ATP molecules through a complex catalytic mechanism. While the nuclear genome encodes the majority of ATP synthase subunits, the mitochondrial genome encodes some of the enzyme's most critical components. The formation of this multi-subunit enzyme is a complex multi-step process regulated at the level of transcription, translation, and assembly. Defects in one or more of these steps can result in decreased...
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ATP Synthase: Structure01:18

ATP Synthase: Structure

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ATP synthase or ATPase is among the most conserved proteins found in bacteria, mammals, and plants. This enzyme can catalyze a forward reaction in response to the electrochemical gradient, producing ATP from ADP and inorganic phosphate. ATP synthase can also work in a reverse direction by hydrolyzing ATP and generating an electrochemical gradient. Different forms of ATP synthases have evolved special features to meet the specific demands of the cell. Based on their specific feature, ATP...
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Chemical Synapses01:26

Chemical Synapses

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Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
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Chemical Synapses01:26

Chemical Synapses

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Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
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Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

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Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
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Myasthenia Gravis: Diagnostic Tests01:15

Myasthenia Gravis: Diagnostic Tests

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Myasthenia gravis is an autoimmune condition affecting neuromuscular transmission, causing generalized weakness in skeletal muscles. Initial diagnoses rely on patients' signs, symptoms, and medical history. The challenge lies in distinguishing myasthenia from other muscular dystrophies. An important diagnostic feature is the significant improvement of symptoms after administering anticholinesterase inhibitors.
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
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Related Experiment Video

Updated: Dec 27, 2025

The Neuromuscular Junction: Measuring Synapse Size, Fragmentation and Changes in Synaptic Protein Density Using Confocal Fluorescence Microscopy
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The Neuromuscular Junction: Measuring Synapse Size, Fragmentation and Changes in Synaptic Protein Density Using Confocal Fluorescence Microscopy

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[Antisynthetase myopathy].

Shigeaki Suzuki1

  • 1Department of Neurology, Keio University School of Medicine.

Rinsho Shinkeigaku = Clinical Neurology
|February 27, 2020
PubMed
Summary

Antisynthetase myopathy, a subtype of inflammatory myopathy, presents with muscle weakness and often interstitial lung disease. Early diagnosis and immunosuppressive therapy show favorable outcomes, but lung disease impacts mortality.

Area of Science:

  • Rheumatology
  • Neurology
  • Immunology

Background:

  • Idiopathic inflammatory myopathies are immune-mediated diseases affecting skeletal muscle and other organs.
  • Current classification relies independently on clinical, pathological, and autoantibody diagnoses.
  • Antisynthetase syndrome is defined by aminoacyl transfer RNA synthetase (ARS) antibodies, myositis, and other systemic features.

Purpose of the Study:

  • To characterize antisynthetase myopathy as a distinct entity within idiopathic inflammatory myopathies.
  • To investigate the clinical, pathological, and serological features of antisynthetase myopathy.
  • To evaluate treatment responses and prognostic factors in antisynthetase myopathy.

Main Methods:

  • A cohort study of 460 patients with idiopathic inflammatory myopathies.
Keywords:
aminoacyl transfer RNA synthetase (ARS)anti-OJ antibodiesautoantibodiesinflammatory myopathiesmuscle pathology

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  • Analysis of muscle biopsy, clinical data, and autoantibody profiles (including anti-ARS and anti-OJ antibodies).
  • Evaluation of diagnostic methods for anti-ARS antibodies, noting limitations of conventional assays for anti-OJ.

    • Main Results:

    • Antisynthetase myopathy was identified in 11% (51/460) of patients.
    • Common manifestations include muscle weakness, dysphagia, and muscle atrophy; perifascicular necrosis is a key pathological finding.
    • Interstitial lung disease occurred in 41 patients and was a significant predictor of mortality.

    Conclusions:

    • Antisynthetase myopathy is a distinct clinical and histological entity within idiopathic inflammatory myopathies.
    • Early diagnosis and combined immunosuppressive therapy lead to favorable outcomes.
    • Interstitial lung disease is a critical comorbidity impacting prognosis in antisynthetase myopathy.