Progranulin in neurodegenerative diseases

被引:0
作者
Harmata, Barbara [1 ]
Jedrzejowska-Szypulka, Halina [1 ]
Pawletko, Katarzyna [1 ]
Lewin-Kowalik, Joanna [1 ]
机构
[1] Slaski Uniwersytet Med Katowicach, Wydzial Lekarski Katowicach, Katedra & Zaklad Fizjol, Ul Medykow 18, PL-40752 Katowice, Poland
来源
POSTEPY HIGIENY I MEDYCYNY DOSWIADCZALNEJ | 2018年 / 72卷
关键词
progranulin; neurodegeneration; frontotemporal dementia; Alzheimer's disease; Parkinson's disease; spinal cord injury; ischemic stroke; toxins; FRONTOTEMPORAL LOBAR DEGENERATION; CELLULAR-LOCALIZATION; GENE-EXPRESSION; MUTATIONS; PROTEIN; BRAIN; NEUROPATHOLOGY; INCLUSIONS; PRECURSOR; SEQUENCE;
D O I
10.5604/01.3001.0012.7836
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
Neurodegenerative diseases as a group of congenital or acquired diseases of the nervous system are currently more common in the aging societies, being one of the most severe diseases faced the healthcare services. Many scientific teams are searching for a better understanding of the mechanisms of these diseases, i.e. their detection, treatment as well as putative prevention methods. Progranulin is one of the twenty-first century discoveries and is a precursor of multifunctional cysteine-rich proteins granulins, which play a crucial role in cell development, cell cycle control, wound healing, inflammatory and cancerogenic processes, neuron development, and neurodegeneration. In 2006, the first mutation in the gene encoding progranulin was discovered as the cause of frontotemporal dementia (FTLD). The human PGRN sequence was first fully characterized on the basis of mRNA isolated from bone marrow cells. Up to now, more than 75 mutations have been reported in patients with various forms of FTLD. All discovered mutations cause the disease either by loss of function of the entire progranulin protein or loss of function of one allele of the PGRN gene. Taking into account the available data regarding PGRN, we would like to collect and systematize the data about progranulin and its putative usefulness as a prognostic factor in some neurodegenerative diseases. The role of progranulin in neurodegenerative diseases is invaluable, not only as a prognostic factor in the presymptomatic stage of the disease but also as a chance for its future therapeutic application in neurodegenerative diseases. Low levels of progranulin were found to correlate with either prodromal symptoms or even asymptomatic stages in patients with established mutations in the progranulin-encoding gene. Research currently in progress provides valuable information about the possibility of this protein being used to improve the diagnosis as well as treatment of different neurodegenerative diseases.
引用
收藏
页码:1052 / 1061
页数:10
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