Vår mitokondrielle stamcelleforskningsgruppe er lokalisert ved Institutt for klinisk medisin (K1), Universitetet i Bergen og er en del av Neuro-SysMed, Senter for fremragende forskning innen nevrologiske lidelser, Nevrologisk avdeling ved Haukeland Universitetssykehus. Vi bruker stamceller transformert fra en pasients egne fibroblaster for å studere mitokondrielle sykdommer forårsaket av POLG-mutasjoner. Målet til forskningsgruppen vår er å gjennomføre iPSC-baserte in vitro modellsystemstudier for å øke forståelsen av mitokondrielle sykdommer, identifisere de underliggende mekanismene til disse vanlige mitokondrielle sykdommene og utvikle en iPSC-basert plattform for å teste terapeutiske midler. Vi har etablert egenskapene og fasilitetene som kreves for omprogrammering og differensiering av iPSC-er, som muliggjør studiet av nevronceller fra pasienter og friske kontroller. Validerte iPSC-er kan differensiere til nevrale stamceller (NSCs) og kompartmentaliserte nevronale subtyper, så vel som astrocytt-/glialcellepopulasjoner. Vi har også nylig utviklet 3D hjerneorganoider for å studere sykdomsmekanismer og teste behandlinger. Vårt brede forskningsmål er å bestemme det biologiske og patologiske grunnlaget for nevrodegenerative sykdommer assosiert med mitokondriell dysfunksjon, og vi tar sikte på å utvikle dette og gjennomføre mer dyptgående molekylære fenotypingsstudier. Siden gruppen også er en del av Neuro-SysMed Center, vil vi bruke vår modell og stamcelletilnærming til å studere andre nevrodegenerative sykdommer som Parkinsons og Alzheimers.

Se også vår gruppenettside: Mitochondrial Stem Cell Research | Mitochondrial Medicine & Neurogenetics (MMN) | UiB

2025

• Xing, Xiaodan; Tang, Chunling; Murdoch, Siofra et al. (2025). Artificial immunofluorescence in a flash: Rapid synthetic imaging from brightfield through residual diffusion. (ekstern lenke)

2024

• Wang, Heling; Siow, Richard; Schmauck-Medina, Tomás Alejandro et al. (2024). Meeting Summary of The NYO3 5th NO-Age/AD Meeting and the 1st Norway-UK Joint Meeting on Aging and Dementia: Recent Progress on the Mechanisms and Interventional Strategies. (ekstern lenke)
• Liang, Xiao (2024). Interplay of mitochondria and diabetes: Unveiling novel therapeutic strategies. (ekstern lenke)
• Chen, Anbin; Yangzom, Tsering; Hong, Yu et al. (2024). Hallmark Molecular and Pathological Features of POLG Disease are Recapitulated in Cerebral Organoids. (ekstern lenke)
• Liang, Xiao (2024). The application of brain organoid for drug discovery in mitochondrial diseases. (ekstern lenke)
• Hong, Yu; Zhang, Zhuoyuan; Yangzom, Tsering et al. (2024). The NAD+ Precursor Nicotinamide Riboside Rescues Mitochondrial Defects and Neuronal Loss in iPSC derived Cortical Organoid of Alpers' Disease. (ekstern lenke)
• Liang, Xiao; Chen, Anbin; Kianian, Atefeh et al. (2024). Activation of Neurotoxic Astrocytes Due to Mitochondrial Dysfunction Triggered by POLG Mutation. (ekstern lenke)

2023

• Kristiansen, Cecilie Katrin; Furriol, Jessica; Chen, Anbin et al. (2023). Deoxyribonucleoside treatment rescues EtBr-induced mtDNA depletion in iPSC-derived neural stem cells with POLG mutations. (ekstern lenke)
• Marjan, Sharika; Yangzom, Tsering; Kristiansen, Cecilie Katrin et al. (2023). Application of Flow Cytometric Analysis for Measuring Multiple Mitochondrial Parameters in 3D Brain Organoids. (ekstern lenke)
• Hong, Yu; Kristiansen, Cecilie Katrin; Chen, Anbin et al. (2023). POLG genotype influences degree of mitochondrial dysfunction in iPSC derived neural progenitors, but not the parent iPSC or derived glia. (ekstern lenke)

2022

• Zhao, Lin; Wang, Han; Liu, Sijia et al. (2022). Inhibition of autophagy reduces the rate of fluoride-induced LS8 apoptosis via regulating ATG5 and ATG7. (ekstern lenke)
• Li, Shaoming; Gao, Ling; Zhang, Weidong et al. (2022). MiR-152-5p suppresses osteogenic differentiation of mandible mesenchymal stem cells by regulating ATG14-mediated autophagy. (ekstern lenke)
• Kristiansen, Cecilie Katrin; Chen, Anbin; Høyland, Lena Elise et al. (2022). Comparing the mitochondrial signatures in ESCs and iPSCs and their neural derivations. (ekstern lenke)
• Chen, Anbin; Kristiansen, Cecilie Katrin; Høyland, Lena Elise et al. (2022). POLG mutations lead to abnormal mitochondrial remodeling during neural differentiation of human pluripotent stem cells via SIRT3/AMPK pathway inhibition. (ekstern lenke)

2021

• Zhi, Yuan; Gao, Ling; Wang, Baisheng et al. (2021). Ferroptosis Holds Novel Promise in Treatment of Cancer Mediated by Non-coding RNAs. (ekstern lenke)
• Liang, Xiao; Chen, Anbin; Kristiansen, Cecilie Katrin et al. (2021). Flow cytometric analysis of multiple mitochondrial parameters in human induced pluripotent stem cells and their neural and glial derivatives. (ekstern lenke)
• Chen, Anbin; Kristiansen, Cecilie Katrin; Hong, Yu et al. (2021). Nicotinamide riboside and metformin ameliorate mitophagy defect in induced pluripotent stem cell-derived astrocytes with POLG mutations. (ekstern lenke)
• Zhao, Lin; Su, Jiali; Liu, Sijia et al. (2021). MAP kinase phosphatase MKP-1 regulates p-ERK1/2 signaling pathway with fluoride treatment. (ekstern lenke)

2020

• Liang, Xiao; Vatne, Guro Helén; Kristiansen, Cecilie Katrin et al. (2020). N-acetylcysteine amide ameliorates mitochondrial dysfunction and reduces oxidative stress in hiPSC-derived dopaminergic neurons with POLG mutation. (ekstern lenke)
• Gao, Ling; Wang, Qibo; Ren, Wen-Hao et al. (2020). The RBP1–CKAP4 axis activates oncogenic autophagy and promotes cancer progression in oral squamous cell carcinoma. (ekstern lenke)
• Balafkan, Novin; Mostafavi, Sepideh; Schubert, Manja et al. (2020). A method for differentiating human induced pluripotent stem cells toward functional cardiomyocytes in 96‐well microplates. (ekstern lenke)
• Hong, Yu; Liang, Kristina Xiao; Gilhus, Nils Erik (2020). AChR antibodies show a complex interaction with human skeletal muscle cells in a transcriptomic study. (ekstern lenke)
• Liang, Xiao; Kristiansen, Cecilie Katrin; Mostafavi, Sepideh et al. (2020). Disease-specific phenotypes in iPSC-derived neural stem cells with POLG mutations. (ekstern lenke)
• Liang, Xiao; Kristiansen, Cecilie Katrin; Vatne, Guro Helén et al. (2020). Patient-specific neural progenitor cells derived from induced pluripotent stem cells offer a promise of good models for mitochondrial disease. (ekstern lenke)

2019

• Zhuoyuan, Zhang; Gao, Zhenjie; Rajthala, Saroj et al. (2019). Metabolic reprogramming of normal oral fibroblasts correlated with increased glycolytic metabolism of oral squamous cell carcinoma and precedes their activation into carcinoma associated fibroblasts. (ekstern lenke)

2018

• Kristiansen, Cecilie Katrin; Bindoff, Laurence; Liang, Xiao (2018). W748S POLG mutation induced mitochondrial changes in hiPSC-differentiated NSCs. (ekstern lenke)
• Kitajima, Kayoko; Das, Ridhima; Liang, Xiao et al. (2018). Isolation and characterization of cells derived from human epithelial rests of Malassez. (ekstern lenke)

2017

• Liang, Xiao; Wei, Zhiyuan; Hu, Deyu et al. (2017). Prevalence of dentin hypersensitivity among the residents of Xi'an city, China. (ekstern lenke)

Se en full oversikt over publikasjoner i Cristin

Publications:

1.     Kristina Xiao Liang (first and leading-corresponding author), Atefeh Kianian, Anbin Chen, Cecilie Katrin Kristiansen, Yu Hong, Jessica Furriol, Lena Elise Høyland, Mathias Ziegler, Torbjørn Kråkenes, Charalampos Tzoulis, Gareth John Sullivan, Laurence A. Bindoff, Cell Stem Cell, in review, doi: https://doi.org/10.1101/2020.12.20.423652, Stem cell derived astrocytes with POLG mutations and mitochondrial dysfunction including abnormal NAD+ metabolism is toxic for neurons

2.     Kristina Xiao Liang (first and leading-corresponding author), Guro Helén Vatne, Cecilie Katrin Kristiansen, Oleksandr Ievglevskyi, Elena Kondratskaya, Joel C. Glover, Anbin Chen, Gareth John Sullivan, Laurence A. Bindoff, Experimental Neurology (2020), https://doi.org/10.1016/j.expneurol.2020.113536. N-acetylcysteine amide ameliorates mitochondrial dysfunction and reduces oxidative stress in hiPSC-derived dopaminergic neurons with POLG mutation.

3.     Kristina Xiao Liang (first and leading-corresponding author), Cecilie Katrin Kristiansen, Sepideh Mostafavi, Guro Helén Vatne, Gina Alien Zanting, Atefeh Kianian, Charalampos Tzoulis, Lena Elise Høyland, Mathias Ziegler, Roberto Megias Pered, Jessica Furriol Zhuoyuan Zhang, Novin Balafkan, Yu Hong, Richard Sillerg, Gareth John Sullivan and Laurence Albert Bindoff, EMBO Mol Med (2020), e12146, Disease‐specific phenotypes in iPSC‐derived neural stem cells with POLG mutations.

4.     X Liang (first and leading-corresponding author), CK Kristiansen, GH Vatne, Y Hong, LA Bindoff, Cell and Tissue Research, 1-16 (2020), Patient-specific neural progenitor cells derived from induced pluripotent stem cells offer a promise of good models for mitochondrial disease.

5.     Novin Balafkan, Sepideh Mostafavi, Manja Schubert, Richard Siller, Kristina Xiao Liang, Gareth Sullivan & Laurence A. Bindoff, Sientific Reports (2020) Method for differentiating human induced pluripotent stem cells toward functional cardiomyocytes in 96-well microplates.

6.     L Gao, Q Wang, W Ren, J Zheng, S Li, Z Dou, X Kong, X Liang (co-corresponding author), K Zhi, Cell death & disease 11 (6), 1-16 (2020), The RBP1-CKAP4 axis activates oncogenic autophagy and promotes cancer progression in oral squamous cell carcinoma.

7.     Lin Zhao, Jiali Su, Sijia Liu, Yang Li, Tao Li, Jianping Ruan, Kristina Xiao Liang (co-corresponding author), Ruizhe Huang, MAP kinase phosphatase MKP-1 regulates p-ERK1/2 signaling pathway with fluoride treatment. BBRC 2020.

8.     Y Hong, X Liang, NE Gilhus, Scientific reports 10 (1), 1-13 (2020), AChR antibodies show a complex interaction with human skeletal muscle cells in a transcriptomic study.

9.     Z Zhang, Z Gao, S Rajthala, D Sapkota, H Dongre, H Parajuli, S Suliman, R Das, L Li, LA Bindoff, DE Costea, X Liang (leading-corresponding author). Cellular and Molecular Life Sciences, 1-19 (2019), Metabolic reprogramming of normal oral fibroblasts correlated with increased glycolytic metabolism of oral squamous cell carcinoma and precedes their activation into carcinoma.

10.  L Gao, ZC Dou, WH Ren, SM Li, X Liang (co-corresponding author), KQ Zhi, Cell death & disease 10 (10), 1-16, (2019), CircCDR1 as upregulates autophagy under hypoxia to promote tumor cell survival via AKT/ERK 1⁄2/mTOR signaling pathways in oral squamous cell carcinomas.

11.  Z Zhang, X Liang, Y Fan, Z Gao, LA Bindoff, DE Costea, L Li, Cell Cycle 18 (9), 949-962 (2019), Fibroblasts rescue oral squamous cancer cell from metformin-induced apoptosis via alleviating metabolic disbalance and inhibiting AMPK pathway.

12.  Q Wang,Y Zhi, W Ren, S Li, Z Dou, X Xing, X Quan, Y Wang, C Jiang, X Liang, L Gao, K Zhi, Journal of cellular physiology 234 (9), 15330-15341 (2019), Suppression of OSCC malignancy by oral glands derived-PIP identified by iTRAQ combined with 2D LC-MS/MS.

13.  K Kitajima, R Das, X Liang, E Neppelberg, AC Johannessen, DE Costea, et al, Odontology 107 (3), 291- 300 (2019), AChR antibodies show a complex interaction with human skeletal muscle cells in a transcriptomic study.

14.  X Liang, Z Wei, D Hu, J Ruan. Acta Odontol Scand. Aug;75(6):387-393 (2017), Prevalence of dentin hypersensitivity among the residents of Xi'an city, China.

15.  A Biddle, L Gammon, X Liang, DE Costea, IC Mackenzie, EBioMedicine (2016); 4: 138–45, Phenotypic plasticity determines cancer stem cell therapeutic resistance in oral squamous cell

16.  N Yang, T Yan, H Zhu, X Liang, L Leiss, PØ Sakariassen, KO Skaftnesmo, B Huang, DE Costea, PØ Enger, X Li, J.J Wang, Transl Med. Oct 4;12:278 (2014), A co-culture model with brain tumorspecific bioluminescence demonstrates astrocyte- induced drug resistance in glioblastoma.

17.  X Liang, KA Graham, AC Johannessen, DE Costea, FH Labeed, Integrative Biology 6 (5), 545- 554 (2014), Human oral cancer cells with increasing tumorigenic abilities exhibit higher effective membrane capacitance.

18.  X Liang, TAH Osman, D Sapkota, E Neppelberg, S Lybak, PG Liavaag, et al., European journal of cancer 50 (18), 3262-3270 (2014), Rapid adherence to collagen IV enriches for tumour initiating cells in oral cancer.

19.  N Yang, H Zhu, T Yan, X Liang, LW Leiss, PØ Enger, X Li, J Wang, Cancer Research 74 (19 Supplement) 2014, A novel model to study the stroma-induced drug resistance in glioblastoma cells.

20.  A Biddle, X Liang, L Gammon, B Fazil, LJ Harper, H Emich, DE Costea, Cancer research 71 (15), 5317-5326 (2011), Cancer stem cells in squamous cell carcinoma switch between two distinct phenotypes that are preferentially migratory or proliferative.

21.  HJ Mulhall, R Abdallat, X Liang, S Fedele, MP Lewis, S Porter, et al., TechConnect Briefs, Volume: 3, Nanotechnology (2010): Bio Sensors, Instruments, Medical, Environment and Energy (2010), Rapid detection of oral cancer: Electrophysiological characterization by eielectrophoresis technology.

22.   Que K, Ruan J, Fan X, Liang X, Hu D. J Clin Periodontol. Jul;37(7):631-7 (2010) A multi-centre and cross-sectional study of dentine hypersensitivity in China.