Rapid detection of GM1 ganglioside in cerebrospinal fluid in dogs with GM1 gangliosidosis using matrix-assisted laser desorption ionization time-of-flight mass spectrometry.

[gm1 gangliosidosis]

The concentration of GM 1 (monosialotetrahexosyl ganglioside ) in cerebrospinal fluid (CSF ) is markedly increased in dogs with GM 1 gangliosidosis due to GM 1 accumulation in the central nervous system and leakage to the CSF . The present study established a rapid and simple method for detection of accumulated GM 1 in the CSF in dogs with GM 1 gangliosidosis using matrix-assisted laser desorption ionization time-of-flight mass spectrometry (MALDI TOF MS ) and discusses the usefulness of this method for the rapid diagnosis and /or high -risk screening of this disease in domestic animals . Cerebrospinal fluid was collected from normal dogs and 4 - to 11 -month -old Shiba dogs with GM 1 gangliosidosis . The MALDI TOF MS analysis was carried out in combination with a special sample plate and a simple desalting step on the plate . Specific signs of GM 1 could be detected in the standard GM 1 solutions at concentrations of 50 nmol /l or more . The signs were also clearly detected in CSF (131 -618 nmol /l ) in affected dogs , but not in normal canine CSF (12 ± 5 nmol /l , mean ± standard deviation ). The results demonstrated that MALDI TOF MS can detect GM 1 accumulated in canine CSF even in the early stage of the disease . In conclusion , the rapid detection of increased CSF GM 1 using MALDI TOF MS is a useful method for diagnosis and /or screening for canine GM 1 gangliosidosis .