Sains Malaysiana 50(5)(2021): 1381-1392

http://doi.org/10.17576/jsm-2021-5005-17

Down Syndrome and Cell Therapy: A Review

(Sindrom Down dan Terapi Sel: Suatu Ulasan)

NORIZAM SALAMT¹, RUSZYMAH HAJI IDRUS¹ & NOOR WAHIDAH MOHD NASRI^1,2*

¹Department of Physiology, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Centre, Bandar Tun Razak, 56000 Cheras, Kuala Lumpur, Malaysia

²Department of Nursing, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Centre, Bandar Tun Razak, 56000 Cheras, Kuala Lumpur, Malaysia

Received: 22 October 2019/Accepted: 28 September 2020

ABSTRACT

Cell therapy has been considered as an alternative treatment for many diseases, including Down syndrome (DS). However, this treatment remained debatable due to insufficient clinical data.  Therefore, this study aims to identify and evaluate studies on the potential of cell therapy in improving the quality of life of DS patients. Relevant English articles and snowball sampling from Science Direct and PubMed (published until August 2019) on the effects of cell therapy on DS was retrieved. Only original articles on the effect of cell therapy in DS patients or Ts65Dn (trisomic) mice were selected. Two independent reviewers reviewed the articles with selective inclusion criteria using a standard data extraction form. Cell therapy showed no significant findings on the physical appearance, cognitive function, social, and behavior skills of DS patients. Interestingly, implantation of murine neural stem cell (mNSC) or murine neural progenitor cell (mNPC) showed better cell survival and response towards brain injury, decrease tau + granules and increase granules density in the dentate gyrus in the trisomic mice. mNSC/mNPC in mice brain was found to be able to migrate to the sites of the injury following chemokine signals and eventually provide neuroprotection and promote axonal growth. To conclude, mNSC/mNPC implantation could be considered as an alternative treatment for DS or DS with early onset of Alzheimer Disease (AD).

Keywords: Cell therapy; Down syndrome; sicca cell therapy; trisomy 21

ABSTRAK

Rawatan terapi sel telah dipertimbangkan sebagai rawatan alternatif pelbagai penyakit, termasuklah Sindrom Down (SD). Walau bagaimanapun, keberkesanan terapi sel masih dibincangkan kerana kekurangan data klinikal. Maka, kami berazam untuk mengenal pasti dan menilai kajian ke atas potensi terapi sel dalam meningkatkan kualiti hidup pesakit SD. Makalah bahasa Inggeris berkaitan dan persampelan snowball atau hand selected daripada Science Direct dan Pubmed (diterbitkan sehingga Ogos 2019) berkaitan kesan terapi sel ke atas SD telah dijalankan. Hanya makalah asal tentang kesan terapi sel pada pesakit SD atau mencit Ts65Dn (trisomi) telah dipilih. Dua orang penilai bebas telah menilai makalah dengan kriteria rangkuman pilihan menggunakan borang pengekstrakan data lazim. Terapi sel menunjukkan keputusan tanpa implikasi terhadap penampilan fizikal, fungsi kognitif dan kemahiran sosial serta tingkah laku pesakit SD. Menariknya, implan sel stem neuron murin (mNSC) atau sel progenitor neuron murin (mNPC) menunjukkan kebolehan sel untuk terus hidup dengan lebih baik dan bertindak balas terhadap kecederaan otak, mengurangkan granul tau+ dan meningkatkan ketumpatan granul di girus dentat pada mencit trisomi. mNSC/mNPC pada otak mencit menunjukkan kebolehan untuk bergerak ke bahagian yang mengalami kecederaan berpandukan isyarat kemokin dan seterusnya melindungi neuron dan menggalakkan pertumbuhan akson. Kesimpulannya, pengimplanan mNSC/mNPC boleh dijadikan sebagai rawatan alternatif untuk pesakit SD atau mengurangkan simptom penyakit Alzhemeir dalam kalangan pesakit SD.

Kata kunci: Sindrom Down; terapi sel; terapi sel sicca; trisomi 21

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*Corresponding author; email: idanasri@ppukm.ukm.edu.my