The Predictive Role of Heat Shock Proteins 27 and 60 in Pediatric Patients with Ataxia Telangiectasia


Abstract views: 63 / PDF downloads: 32

Authors

DOI:

https://doi.org/10.5152/eurjther.2020.20019

Keywords:

Ataxia telangiectasia, heat shock protein 27, heat shock protein 60, chaperone, children

Abstract

Objective: This study aimed to measure the levels of heat shock proteins (HSP) 27 and 60 as mediators for hypoxia and tissue injury in pediatric patients with ataxia telangiectasia (AT). Another aim was to determine the prognostic role of HSP 27 and 60 in AT.
Methods: This study analyzed the data of AT patients (n=15) aged 4-16 years and of age-matched healthy controls (n=20). HSP 27 and 60 levels were measured from serum samples of AT patients using an enzyme-linked immunosorbent assay and compared with those of healthy controls.
Results: Serum levels of HSP 27 and 60 were higher in patients with AT than in age-matched healthy controls (p=0.010 and p=0.000, respectively).
Conclusion: In AT patients, levels of HSP 27 and 60 are increased to prevent tissue injury; therefore, treatment targeting HSPs may prevent disease progression and development of secondary malignancy in this patient population.

Metrics

Metrics Loading ...

References

McKinnon PJ. ATM and ataxia telangiectasia. EMBO Rep 2004; 5: 772-6.

Chun HH, Gatti RA. Ataxia-telangiectasia, an evolving phenotype. DNA Repair (Amst) 2004; 3: 1187-96.

Humar B, Muller H, Scott RJ. Cell cycle dependent DNA break increase in ataxia telangiectasia lymphoblasts after radiation exposure. Mol Pathol 2001; 54: 347-50.

Sharp FR, Massa SM, Swanson RA. Heat-shock protein protection. Trends Neurosci 1999; 22: 97-9.

Perschinka H, Mayr M, Millonig G, Mayerl C, Van der Zee R, Morrison SG, et al. Cross-reactive B-cellepitopes of microbial and human heat shock protein 60/65 in atherosclerosis. Arterioscler Thromb Vasc Biol 2003; 23: 1060-5.

Magen D, Georgopoulos C, Bross P, Ang D, Segev Y, Goldsher D, et al. Mitochondrial hsp60 chaperonopathy causes an autosomal-recessive neurodegenerative disorder linked to brain hypomyelination and leukodystrophy. Am J Hum Genet 2008; 83: 30-42.

Trouillas P, Takayanagi T, Hallett M, Currier RD, Subramony SH, Wessel K, et al. International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neurology. J Neurol Sci 1997; 145: 205-11.

Multhoff G. Heat-shock protein 70 (HSP 70): membrane location, export and immunological relevance. Methods 2007; 43: 229-37.

Bukau B, Horwich AL. The HSP 70 and HSP 60 chaperone machines. Cell 1998; 92: 351-66.

Franklin TB, Krueger-Naug AM, Clarke DB, Arrigo AP, Currie RW. The role of heat shock proteins HSP 70 and HSP 27 in cellular protection of the central nervous system. Int J Hyperthermia 2005; 21: 379-92.

Di Domenico F, Sultana R, Tiu GF, Scheff NN, Perluigi M, Cini C, et al.Protein levels of heat-shock proteins 27, 32, 60, 70, 90 and thioredoxin-1 in amnestic mild cognitive impairment: an investigation on the role of cellular stress response in the progression of Alzheimer disease. Brain Res 2010; 1333: 72-81.

Raaphorst GP, Azzam EI. Hyperthermia enhances X ray cell killing in normal and homozygous and heterozygous ataxia telangiectasia human cells. Int J Radiat Oncol Biol Phys 1985; 11: 855-9.

Akbar MT, Lundberg AM, Liu K, Vidyadaran S, Wells KE, Dolatshad H, et al. The neuroprotective effects of heat shock protein 27 overexpression in transgenic animals against kainate-induced seizures and hippocampal cell death. J Biol Chem 2003; 278: 19956-65.

Lai Y, Stange C, Wisniewski SR, Adelson PD, Janesko-Feldman KL, Brown DS, et al. Mitochondrial heat shock protein 60 is increased in cerebrospinal fluid following pediatric traumatic brain injury. Dev Neurosci 2006; 28: 336-41.

Yang QW, Li JC, Lu FL, Wen AQ, Xiang J, Zhang LL, et al. Upregulated expression of toll-like receptor 4 in monocytes correlates with severity of acute cerebral infarction. J Cereb Blood Flow Metab 2008; 28: 1588-96.

Xi ZQ, Sun JJ, Wang XF, Li MW, Liu XZ, Wang LY, et al. HSPBAP1 is found extensively in the anterior temporal neocortex of patients with intractable epilepsy. Synapse 2007; 61: 741-7.

Chen W, Kuizon S, Chiou BL, Bolton DC, Pullarkat RK, Junaid MA. Differential expression of small heat shock protein 27 (HSP 27) in Ataxia telangiectasia brains. Neurochem Res 2009; 34: 1658-67.

Downloads

Published

2020-12-01

How to Cite

Özçelik, A., Perk, P., Özyurt, B., & Kılıç, B. D. (2020). The Predictive Role of Heat Shock Proteins 27 and 60 in Pediatric Patients with Ataxia Telangiectasia. European Journal of Therapeutics, 26(4), 287–290. https://doi.org/10.5152/eurjther.2020.20019

Issue

Section

Original Articles