HPD degradation regulated by the TTC36-STK33-PELI1 signaling axis induces tyrosinemia and neurological damage

HPD degradation regulated by the TTC36-STK33-PELI1 signaling axis induces tyrosinemia and neurological damage

Decreased expression of 4-hydroxyphenylpyruvic acid dioxygenase (HPD) has been linked to tyrosinemia, yet the mechanism underlying the regulation of HPD expression is largely unknown. Here the authors demonstrate that molecular chaperone TTC36, which is highly expressed in liver, is associated with...

Descripción completa

Guardado en:
Detalles Bibliográficos
Autores principales: Yajun Xie, Xiaoyan Lv, Dongsheng Ni, Jianing Liu, Yanxia Hu, Yamin Liu, Yunhong Liu, Rui Liu, Hui Zhao, Zhimin Lu, Qin Zhou
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2019
Materias:
Science
Q
Acceso en línea:https://doaj.org/article/023dc676f75144518fa15b54efb57586
Etiquetas: Agregar Etiqueta
Sin Etiquetas, Sea el primero en etiquetar este registro!
id oai:doaj.org-article:023dc676f75144518fa15b54efb57586
record_format dspace
spelling oai:doaj.org-article:023dc676f75144518fa15b54efb575862021-12-02T17:31:22ZHPD degradation regulated by the TTC36-STK33-PELI1 signaling axis induces tyrosinemia and neurological damage10.1038/s41467-019-12011-02041-1723https://doaj.org/article/023dc676f75144518fa15b54efb575862019-09-01T00:00:00Zhttps://doi.org/10.1038/s41467-019-12011-0https://doaj.org/toc/2041-1723Decreased expression of 4-hydroxyphenylpyruvic acid dioxygenase (HPD) has been linked to tyrosinemia, yet the mechanism underlying the regulation of HPD expression is largely unknown. Here the authors demonstrate that molecular chaperone TTC36, which is highly expressed in liver, is associated with HPD and reduces the binding of protein kinase STK33 to HPD, thereby inhibiting STK33-mediated HPD T382 phosphorylation.Yajun XieXiaoyan LvDongsheng NiJianing LiuYanxia HuYamin LiuYunhong LiuRui LiuHui ZhaoZhimin LuQin ZhouNature PortfolioarticleScienceQENNature Communications, Vol 10, Iss 1, Pp 1-12 (2019)
institution DOAJ
collection DOAJ
language EN
topic Science
Q
spellingShingle Science
Q
Yajun Xie
Xiaoyan Lv
Dongsheng Ni
Jianing Liu
Yanxia Hu
Yamin Liu
Yunhong Liu
Rui Liu
Hui Zhao
Zhimin Lu
Qin Zhou
HPD degradation regulated by the TTC36-STK33-PELI1 signaling axis induces tyrosinemia and neurological damage
description Decreased expression of 4-hydroxyphenylpyruvic acid dioxygenase (HPD) has been linked to tyrosinemia, yet the mechanism underlying the regulation of HPD expression is largely unknown. Here the authors demonstrate that molecular chaperone TTC36, which is highly expressed in liver, is associated with HPD and reduces the binding of protein kinase STK33 to HPD, thereby inhibiting STK33-mediated HPD T382 phosphorylation.
format article
author Yajun Xie
Xiaoyan Lv
Dongsheng Ni
Jianing Liu
Yanxia Hu
Yamin Liu
Yunhong Liu
Rui Liu
Hui Zhao
Zhimin Lu
Qin Zhou
author_facet Yajun Xie
Xiaoyan Lv
Dongsheng Ni
Jianing Liu
Yanxia Hu
Yamin Liu
Yunhong Liu
Rui Liu
Hui Zhao
Zhimin Lu
Qin Zhou
author_sort Yajun Xie
title HPD degradation regulated by the TTC36-STK33-PELI1 signaling axis induces tyrosinemia and neurological damage
title_short HPD degradation regulated by the TTC36-STK33-PELI1 signaling axis induces tyrosinemia and neurological damage
title_full HPD degradation regulated by the TTC36-STK33-PELI1 signaling axis induces tyrosinemia and neurological damage
title_fullStr HPD degradation regulated by the TTC36-STK33-PELI1 signaling axis induces tyrosinemia and neurological damage
title_full_unstemmed HPD degradation regulated by the TTC36-STK33-PELI1 signaling axis induces tyrosinemia and neurological damage
title_sort hpd degradation regulated by the ttc36-stk33-peli1 signaling axis induces tyrosinemia and neurological damage
publisher Nature Portfolio
publishDate 2019
url https://doaj.org/article/023dc676f75144518fa15b54efb57586
work_keys_str_mv AT yajunxie hpddegradationregulatedbythettc36stk33peli1signalingaxisinducestyrosinemiaandneurologicaldamage
AT xiaoyanlv hpddegradationregulatedbythettc36stk33peli1signalingaxisinducestyrosinemiaandneurologicaldamage
AT dongshengni hpddegradationregulatedbythettc36stk33peli1signalingaxisinducestyrosinemiaandneurologicaldamage
AT jianingliu hpddegradationregulatedbythettc36stk33peli1signalingaxisinducestyrosinemiaandneurologicaldamage
AT yanxiahu hpddegradationregulatedbythettc36stk33peli1signalingaxisinducestyrosinemiaandneurologicaldamage
AT yaminliu hpddegradationregulatedbythettc36stk33peli1signalingaxisinducestyrosinemiaandneurologicaldamage
AT yunhongliu hpddegradationregulatedbythettc36stk33peli1signalingaxisinducestyrosinemiaandneurologicaldamage
AT ruiliu hpddegradationregulatedbythettc36stk33peli1signalingaxisinducestyrosinemiaandneurologicaldamage
AT huizhao hpddegradationregulatedbythettc36stk33peli1signalingaxisinducestyrosinemiaandneurologicaldamage
AT zhiminlu hpddegradationregulatedbythettc36stk33peli1signalingaxisinducestyrosinemiaandneurologicaldamage
AT qinzhou hpddegradationregulatedbythettc36stk33peli1signalingaxisinducestyrosinemiaandneurologicaldamage
_version_ 1718380593436164096

Ejemplares similares