Jorge-Finnigan Ana, Kleppe Rune, Jung-Kc Kunwar, Ying Ming, Marie Michael, Rios-Mondragon Ivan, Salvatore Michael F, Saraste Jaakko, Martinez Aurora
From the Department of Biomedicine, University of Bergen, Jonas Lies vei 91, 5009 Bergen, Norway; K. G. Jebsen Centre for Neuropsychiatric Disorders, Jonas Lies vei 91, 5009 Bergen, Norway.
From the Department of Biomedicine, University of Bergen, Jonas Lies vei 91, 5009 Bergen, Norway; K. G. Jebsen Centre for Neuropsychiatric Disorders, Jonas Lies vei 91, 5009 Bergen, Norway.
J Biol Chem. 2017 Aug 25;292(34):14092-14107. doi: 10.1074/jbc.M116.762344. Epub 2017 Jun 21.
Tyrosine hydroxylase (TH) catalyzes the conversion of l-tyrosine into l-DOPA, which is the rate-limiting step in the synthesis of catecholamines, such as dopamine, in dopaminergergic neurons. Low dopamine levels and death of the dopaminergic neurons are hallmarks of Parkinson's disease (PD), where α-synuclein is also a key player. TH is highly regulated, notably by phosphorylation of several Ser/Thr residues in the N-terminal tail. However, the functional role of TH phosphorylation at the Ser-31 site (THSer(P)-31) remains unclear. Here, we report that THSer(P)-31 co-distributes with the Golgi complex and synaptic-like vesicles in rat and human dopaminergic cells. We also found that the TH microsomal fraction content decreases after inhibition of cyclin-dependent kinase 5 (Cdk5) and ERK1/2. The cellular distribution of an overexpressed phospho-null mutant, TH1-S31A, was restricted to the soma of neuroblastoma cells, with decreased association with the microsomal fraction, whereas a phospho-mimic mutant, TH1-S31E, was distributed throughout the soma and neurites. TH1-S31E associated with vesicular monoamine transporter 2 (VMAT2) and α-synuclein in neuroblastoma cells, and endogenous THSer(P)-31 was detected in VMAT2- and α-synuclein-immunoprecipitated mouse brain samples. Microtubule disruption or co-transfection with α-synuclein A53T, a PD-associated mutation, caused TH1-S31E accumulation in the cell soma. Our results indicate that Ser-31 phosphorylation may regulate TH subcellular localization by enabling its transport along microtubules, notably toward the projection terminals. These findings disclose a new mechanism of TH regulation by phosphorylation and reveal its interaction with key players in PD, opening up new research avenues for better understanding dopamine synthesis in physiological and pathological states.
酪氨酸羟化酶(TH)催化L-酪氨酸转化为L-多巴,这是多巴胺能神经元中儿茶酚胺(如多巴胺)合成的限速步骤。多巴胺水平降低和多巴胺能神经元死亡是帕金森病(PD)的标志,其中α-突触核蛋白也是关键因素。TH受到高度调节,特别是通过N端尾部几个丝氨酸/苏氨酸残基的磷酸化。然而,TH在丝氨酸31位点(THSer(P)-31)磷酸化的功能作用仍不清楚。在这里,我们报告THSer(P)-31在大鼠和人类多巴胺能细胞中与高尔基体复合体和突触样小泡共分布。我们还发现,细胞周期蛋白依赖性激酶5(Cdk5)和ERK1/2受到抑制后,TH微粒体部分含量降低。过表达的磷酸化缺失突变体TH1-S31A的细胞分布局限于神经母细胞瘤细胞的胞体,与微粒体部分的结合减少,而磷酸化模拟突变体TH1-S31E则分布于整个胞体和神经突。TH1-S31E在神经母细胞瘤细胞中与囊泡单胺转运体2(VMAT2)和α-突触核蛋白相关,并且在VMAT2和α-突触核蛋白免疫沉淀的小鼠脑样本中检测到内源性THSer(P)-31。微管破坏或与PD相关突变α-突触核蛋白A53T共转染导致TH1-S31E在细胞胞体中积累。我们的结果表明,丝氨酸31磷酸化可能通过使其沿微管运输,特别是向投射末端运输来调节TH的亚细胞定位。这些发现揭示了TH磷酸化调节的新机制,并揭示了其与PD关键因素的相互作用,为更好地理解生理和病理状态下的多巴胺合成开辟了新的研究途径。