TY  -  
T1  - Deficiency of the ER-stress-regulator MANF triggers progressive outer hair cell death and hearing loss 
SN  -  /  
UR  - http://hdl.handle.net/10138/314197 
T3  -  
A1  - Herranen, Anni; Ikäheimo, Kuu; Lankinen, Tuuli; Pakarinen, Emmi; Fritzsch, Bernd; Saarma, Mart; Lindahl, Maria; Pirvola, Ulla 
A2  -  
PB  -  
Y1  - 2020 
LA  - eng 
AB  - The non-conventional neurotrophic factor mesencephalic astrocyte-derived neurotrophic factor (MANF) is an endoplasmic reticulum (ER)-resident protein that promotes ER homeostasis. MANF has a cytoprotective function, shown in the central nervous system neurons and pancreatic beta cells. Here, we report that MANF is expressed in the hair cells and neurons and in selected non-sensory cells of the cochlea and that Manf inactivation triggers upregulation of the ER chaperones in these cells. However, ... 
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IS  -  
SP  -  
OP  -  
KW  - UNFOLDED PROTEIN RESPONSE; NEUROTROPHIC FACTOR MANF; ENDOPLASMIC-RETICULUM; LOCALIZATION; DEAFNESS; MODEL; MICE; 1182 Biochemistry, cell and molecular biology 
N1  -   
PP  -  
ER  -