Results 131 to 140 of about 41,178 (242)

Ubiquitination of ACSL4 by Parkin Suppresses Ferroptosis and Rescues Glucocorticoid‐Induced Bone Loss

Advanced Science, EarlyView.
GCs reduce Parkin, leading to ACSL4 accumulation and PUFA‐phospholipid‐driven ferroptosis in BMSCs, which impairs osteogenesis and promotes adipogenesis, causing GIOP. Parkin restoration (via OE‐Parkin or Parkin‐LNP@DSS6) ubiquitinates and degrades ACSL4, inhibiting ferroptosis, rescuing bone formation, and rescues GIOP bone loss.
Li‐jiang Han, Jian‐sen Miao, Yi‐feng Shi, Yi‐ting Tu, Yu‐zhe Lin, Lin‐Jie Chen, Bing‐hao Lin, Hua‐lin Li, Xuan‐qi Zheng, Hong‐qiang Wu, Hai‐xiao Liu, Lei Yang, Ai‐min Wu, Zhen Lin, Jian Xiao, Gang Zheng, Chen Jin   +16 more
wiley   +1 more source

A Four-Year Prospective Pilot Study of Newborn Screening for Late-Onset Proximal Urea-Cycle Disorders in Hyogo Prefecture in Japan. [PDF]

Int J Neonatal Screen
Lee T, Matsui M, Yokoyama Y, Bo R, Awano H, Kataoka D, Ueda M, Minato T, Kobayashi H, Hasegawa Y, Murayama K, Takeshima Y.   +11 more
europepmc   +1 more source

PEAR1 Promotes Glucose Metabolism Reprogramming in Sepsis‐Associated Acute Lung Injury via AARS1‐Mediated HIF‐1α Lactylation

Advanced Science, EarlyView.
This study revealed that a PEAR1/HIF‐1α/ glycolysis/lactate/H3K18la positive feedback loop in PMVECs that drives the development of S‐ALI. Mechanistically, PEAR1 mediates the binding of HIF‐1α to AARS1, leading to the lactylation of HIF‐1α, the primary lactylation site of which is K172.
Shuai Li, Zongqing Lu, Pu Fang, Qingqing Pang, Xianghui Wu, Bo Wang, Min Liang, Jingjing Pan, Haobo Kong, Haijian Cai, Juanjuan Li, Jiaying Kang, Dongsheng Wang, Dunling Xia, Mengmeng Xu, Qinghai You   +15 more
wiley   +1 more source

Long-Term Efficacy and Safety of Glycerol Phenylbutyrate in Japanese Patients With Urea Cycle Disorders: Results From a Phase 3 Switch-Over and 12-Month Extension Study. [PDF]

JIMD Rep
Wada Y, Furujo M, Kashimada K, Hamazaki T, Nyuzuki H, Ichimoto K, Kakiuchi T, Matsumoto S, Watanabe Y, Ono C, Shimizu T, Furukawa H, Nakamura K.   +12 more
europepmc   +1 more source

Fifteen years of urea cycle disorders brain research: Looking back, looking forward. [PDF]

Anal Biochem, 2022
Sen K, Whitehead M, Castillo Pinto C, Caldovic L, Gropman A.   +4 more
europepmc   +1 more source

Disrupting Helicobacter pylori Iron Homeostasis With Bismuth Nanodrug‐Mediated Nutritional Trap for Targeted Gastric Infection Therapy

Advanced Science, EarlyView.
A “nutritional trap” strategy centered on disrupting bacterial iron homeostasis is proposed. The synthesized Bi‐TP@FU (TBF) can target Helicobacter pylori, interfere with the bacterial iron uptake and utilization processes to disrupt its iron homeostasis, induce bacterial death, and meanwhile preserve the balance of the intestinal flora.
Tianye Fang, Jinzhe Tong, Feng Feng, Cong Liu, Chang Shu, Jiaying Zhu, Shibo Zhang, Shuyue Deng, Wanchao Zuo, Yuhan Song, Jun Yang, Yanmin Ju, Yingying Xing, Jianjun Dai   +13 more
wiley   +1 more source

Prevalence of fibrosis in hepatic explants and biopsies from individuals with urea cycle disorders. [PDF]

Mol Genet Metab
Ali S, Nisar A, Zhang A, Nagamani S, Aceves-Ewing NM, Rawls B, Quan T, Enns G, Goss J, Leung DH, Shneider BL, Jain S, Hazard FK, Schady D, Burrage LC.   +14 more
europepmc   +1 more source

Targeted Degradation of Picornaviral 3C Protease via PROTACs Confers High Barrier to Viral Resistance and Broad‐Spectrum Antiviral Activity

Advanced Science, EarlyView.
This study reports D34 as the first PROTAC degrader that targets the 3C protease of picornaviruses. D34 effectively degrades EV71 3C protease via the ubiquitin‐proteasome pathway. More importantly, D34 exhibits a high resistance barrier and shows broad‐spectrum antiviral activity against multiple picornaviruses, highlighting its potential as a novel ...
Weilong Deng, Junyu Chen, Yingyue Pang, Yuanyuan Zhang, Siqian Chen, Guoliang You, Xiaoman Tian, Zhongxin Xu, Jing Wang, Luqing Shang   +9 more
wiley   +1 more source

Impact of glycerol phenylbutyrate on biochemistry and outcomes in paediatric patients with urea cycle disorders: a multicentre case series from Saudi Arabia. [PDF]

Orphanet J Rare Dis
Hejazi R, Alghamdi TH, Salih R, Naeim Y, Althiyab H, AlAnzi T, Mohamed S, Alfadhel M, ALTassan RS, Al-Hassnan ZN, AlSayed M.   +10 more
europepmc   +1 more source

Liver Transplantation in Children with Urea Cycle Disorders: The Importance of Minimizing Waiting Time. [PDF]

Liver Transpl, 2021
Ziogas IA, Wu WK, Matsuoka LK, Pai AK, Hafberg ET, Gillis LA, Morgan TM, Alexopoulos SP.   +7 more
europepmc   +1 more source