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

Muscle Nerve. 2017 Mar;55(3):430-432. doi: 10.1002/mus.25438. Epub 2016 Nov 29.


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.

Keywords: ALS; Agrin; LRP4; amyotrophic lateral sclerosis; antibodies; low-density lipoprotein-related receptor protein 4.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Age Factors
  • Agrin / immunology*
  • Amyotrophic Lateral Sclerosis / blood*
  • Autoantibodies / blood*
  • Enzyme-Linked Immunosorbent Assay
  • Female
  • Humans
  • Lipoproteins, LDL / immunology*
  • Male
  • Sex Factors


  • Agrin
  • Autoantibodies
  • Lipoproteins, LDL