Agrin and low-density lipoprotein–related receptor protein 4 antibodies in amyotrophic lateral sclerosis patients

Michael H. Rivner, Siyang Liu, Brandy Quarles, Brandi Fleenor, Chengyong Shen, Jinxiu Pan, Lin Mei

Research output: Contribution to journalArticle

10 Scopus citations


Introduction: The prevalence and characteristics of agrin and low-density lipoprotein–related receptor protein 4 (LRP4) antibody-positive amyotrophic lateral sclerosis (ALS) patients were studied. Methods: We tested 82 ALS patients and 59 controls for agrin and LRP4 antibodies using enzyme-linked immunoassay (ELISA). Results: We found that 13.8% of ALS patients had agrin antibodies, and 9.8% had LRP4 antibodies. Women with ALS are twice as likely as men to have antibodies. Agrin-positive ALS patients are younger than agrin-negative ALS patients. Conclusions: Antibodies to agrin and LRP4 are found in ALS patients. It must be determined whether these antibodies are pathogenic. Because antibody-positive patients have upper as well as lower motor neuron findings, the antibodies' effects cannot be explained solely by their actions at the neuromuscular junction. A breakdown in interneuronal signaling may be the cause of ALS. Further research is needed to resolve this question. Muscle Nerve, 2016 Muscle Nerve 55: 430–432, 2017.

Original languageEnglish (US)
Pages (from-to)430-432
Number of pages3
JournalMuscle and Nerve
Issue number3
StatePublished - Mar 1 2017



  • ALS
  • Agrin
  • LRP4
  • amyotrophic lateral sclerosis
  • antibodies
  • low-density lipoprotein–related receptor protein 4

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)

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