The Expression And Significance Of HAP1in Peripheral Lymphocytes In Patients With ALS

Objective To investigate the expression and variation of huntingtin-associated protein-1(HAP1) in peripheral blood lymphocytes in the patients with amyotrophic lateral sclerosis (ALS) and explore the correlation between HAP1expression and the courses of disease.Methods Collected20patients with suspected or confirmed diagnosed ALS from Xiangya Hospital, Central South University from March1,2012to December31,2012. Two control groups were included, ND group (control with other Neurological disease) and NND group (control with non-Neurological disease). The ND group including multiple sclerosis, myasthenia gravis, Parkinson’s disease patients (n=6per case). The peripheral blood lymphocytes were seperated and then protein and RNA were extracted. Real-time fluorescent quantitative PCR and Western blot were used to detect mRNA and protein expression of HAP1in each group, as well as in the expression change in the patients with different courses.Results The three groups had no statistically significant in the age and gender (P>0.05). Western-blot analysis showed that HAP1protein expression exist in peripheral blood lymphocytes. Real-time quantitative PCR and Western-blot analysis showed that HAP1mRNA and protein expression in peripheral blood lymphocyte of ALS patients were significantly higher than ND and NND group (P<0.05). The HAP1expressions between the ND and NND group had no significant difference (P>0.05). HAP1mRNA and protein expression in ALS patients with a duration more than2years were significantly higher in patients with a duration between1and2years and less than2years groups (P<0.05). And this expression changes and the course of ALS patients were significantly positive correlation. The correlation coefficients were r=0.768and r=0.733(P<0.05), which suggested that with the extension of the course of ALS patients, HAP1protein and mRNA expression in peripheral blood lymphocytes were raised.Conclusion The HAP1protein expression in the people peripheral blood lymphocytes was exist. HAP1protein and mRNA expression in peripheral blood of ALS patients were higher than the normal people and patients with other neurological disorders; HAP1protein and mRNA expression were raised with the extension course of disease. HAP1is an important protein involved in axon transport. It may change expression in ALS pathogenesis and involved in ALS axonal transport disorder. It may become a potential biological markers of ALS.