A possible association of responsiveness to adrenocorticotropic hormone with specific GRIN1 haplotypes in infantile spasms

Dev Med Child Neurol. 2010 Nov;52(11):1028-32. doi: 10.1111/j.1469-8749.2010.03746.x. Epub 2010 Aug 16.

Abstract

Aim: Adrenocorticotropic hormone (ACTH) has been used as the major therapy for infantile spasms since 1958 because it effectively suppresses seizures; it also normalizes the electroencephalogram in the short-term treatment of infantile spasms. G protein-regulated inducer of neurite outgrowth 1 (GRIN1, also known as N-methyl-D-aspartate receptor 1, NMDAR1), a glutamate receptor, is the main component of functional N-methyl-D-aspartic acid receptors that are involved in the glucocorticoid-induced neuronal damage. Thus, it may be a candidate gene to be tested for responsiveness to ACTH in infantile spasms. In the present study, polymorphisms in the GRIN1 gene in infantile spasms were investigated using a case-control design.

Method: Twelve single nucleotide polymorphisms in the GRIN1 gene were genotyped in a Chinese case-control set consisting of 97 unrelated patients with infantile spasms (60 males, 37 females; mean age 6.4 mo, SD 2.7) and 96 healthy individuals (63 males, 33 females; mean age 7.3 mo, SD 3.8). Association analysis was performed on the genotyped data.

Results: Five estimated haplotypes with a frequency of more than 3% were detected. Results of the study showed that responsiveness to treatment with ACTH in homozygous carriers of the CTA haplotype was higher than that in heterozygous carriers and non-carriers (p=0.022). Furthermore, CTG, a rare haplotype, was strongly associated with infantile spasms (p=0.013).

Interpretation: The results suggest that haplotypes of GRIN1 may influence responsiveness to ACTH. The findings necessitate further study for confirmation.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenocorticotropic Hormone / therapeutic use*
  • Carrier Proteins / genetics*
  • Case-Control Studies
  • Female
  • Gene Frequency
  • Genome-Wide Association Study
  • Haplotypes / genetics*
  • Humans
  • Infant
  • Male
  • Nerve Tissue Proteins / genetics*
  • Polymorphism, Single Nucleotide / genetics*
  • Receptors, N-Methyl-D-Aspartate / genetics*
  • Spasms, Infantile / drug therapy
  • Spasms, Infantile / genetics*
  • Treatment Outcome

Substances

  • Carrier Proteins
  • GRIN1 protein, human
  • Nerve Tissue Proteins
  • Receptors, N-Methyl-D-Aspartate
  • Adrenocorticotropic Hormone