The characterisation of molecular forms of acetylcholinesterase in Hirschsprung's disease

Aganglionic rectal tissue from patients with Hirschsprung's disease contains four forms of acetylcholinesterase; the major component has a sedimentation coefficient in the region of 10.0S. Results from gel filtration confirm these findings and, when used in conjunction with the sedimentation data, allow the determination of the molecular mass of these forms. The four species of acetylcholinesterase include: monomer, G1, 74 kDa dimer, G2, 131 kDa; tetramer G4, 275 kDa and an asymmetric form, A12, 811 kDa. Evidence is provided which shows that the major form, G4 interacts with the detergent Triton X-100. Selective measurement of G4-AChE using a suitable assay may provide the basis for improving the existing means of diagnosis of Hirschsprung's disease.