Results 61 to 70 of about 1,507,605 (275)

Management of acute metabolic decompensation in maple syrup urine disease: guidance based on international clinical practice

Orphanet Journal of Rare Diseases
Background Maple syrup urine disease (MSUD) is an autosomal recessive inborn error of metabolism caused by a deficiency of branched-chain ketoacid dehydrogenase, the enzyme involved in the second step of branched-chain amino acid catabolism. Of the three
Aude Servais, Marie Therese Abi-Wardé, Jean-Baptiste Arnoux, Alberto Burlina, Francois Feillet, Vincenzo Giordano, Nicola Longo, Sufin Yap   +7 more
doaj   +1 more source

TRAIP Mediates Alcohol‐Induced Liver Injury through Regulating β‐catenin Ubiquitin Degradation via Direct Interaction

Advanced Science, EarlyView.
This study reveals that NF‐κB1‐driven TRAIP upregulation in ALD correlates with disease severity. Mechanistically, TRAIP directly binds β‐catenin via its CC domain and promotes its K48‐linked ubiquitination and degradation, which is independent of the GSK3β/β‐TrCP pathway.
Zhan Wu, Mingjiang Liu, Lijuan Liao, Zhaohong Mo, Wenli Xu, Linfeng Mao, Yubing Chen, Qiuli Xie, Ming Yao, Shilian Chen, Stephen Tomlinson, Sebastian Mueller, Songqing He, Guandou Yuan   +13 more
wiley   +1 more source

O significado prognóstico dos valores séricos do fator de crescimento do tipo insulina 1 em pacientes admitidos por descompensação aguda da cirrose [PDF]

, 2014
Dissertação (mestrado profissional) - Universidade Federal de Santa Catarina, Centro de Ciências da Saúde, Programa de Pós-Graduação em Cuidados Intensivos e Paliativos, Florianópolis, 2014.Introdução: Níveis reduzidos do fator de crescimento do tipo ...
Colombo, Bruno da Silveira
core  

A Novel Pak1 Activator Ameliorates ER Stress for HFpEF Therapy

Advanced Science, EarlyView.
Chronic metabolic stress is a major contributor to HFpEF progression. Under prolonged metabolic stress, Pak1 activity becomes impaired, contributing to disrupted ER proteostasis, cardiomyocyte apoptosis, fibrosis, and diastolic dysfunction. Mechanistically, Pak1 overexpression activates the ERK1/2–MNK1–eIF4E signaling axis, promotes translational ...
Honglin Xu, Hongyuan Zhang, Tayyiba Azam, Susanne S. Hille, Yingjuan Liu, Min Zi, Jiaqing Lang, He Yu, Yujia Cao, Bernard D. Keavney, Tamer M. A. Mohamed, Jessica M. Miller, Riham R. E. Abouleisa, Ming Xu, Elizabeth J. Cartwright, Oliver J. Müller, Ming Lei, Xin Wang   +17 more
wiley   +1 more source

Ascetic Soluble CD206 and Serum Interleukin-4 Can Predict Mortality in Acute Decompensated and Acute-on-Chronic Liver Failure

Российский журнал гастроэнтерологии, гепатологии, колопроктологии
Aim. Acute-on-chronic liver failure (ACLF) is the most severe form of acute decompensation with intense systemic inflammation and immune dysfunction. IL-4 polarizes macrophages toward the M2 phenotype.
S. A. Lashen, P. Salem, E. ibrahim, D. Abd Elmoaty, W. I. Yousif   +4 more
doaj   +1 more source

Ninety-day survival curves according to previous acute decompensation.

, 2016
(A) Without previous AD vs. with previous AD and (B) without previous AD vs. AD more than 1 year prior vs. AD within 1 year.
Byung Seok Lee (526084), Hyoung Su Kim (847068), Hyung Joon Yim (388116), Dong Hyun Sinn (654999), Young Kul Jung (847063), Seong Woo Nam (847069), Do Seon Song (847059), Jae Young Jang (578041), Jin Mo Yang (847072), Tae Yeob Kim (708864), Joo Hyun Sohn (708865), Heon Ju Lee (847073), Sang Gyune Kim (578046), Dong Joon Kim (280038), Tae Hun Kim (30227), Hee Bok Chae (847071), Ki Tae Suk (282701), Chang Hyeong Lee (847064), Chang H. Kim (204892), Chang Wook Kim (847062), Eun Hee Choi (131957), Jeong Han Kim (847065), Young Seok Kim (578047), Eileen L. Yoon (847061), Hyeok Choon Kwon (847070), Hee Yeon Kim (847060), Soon Koo Baik (693382), Seung Ha Park (847074), Sang Soo Lee (511686), Sung Eun Kim (215879), Byung Hoon Han (847075), Jeong Ill Suh (847067), Won Hyeok Choe (847066)   +32 more
core   +1 more source

TECTB Variants Reveal Tectorial Membrane Vulnerability in Dominant Non‐Syndromic Hearing Loss

Advanced Science, EarlyView.
TECTB is a non‐collagenous protein of the tectorial membrane – an extracellular matrix of the cochlea. This study identifies dominant missense variants in TECTB linked to human hereditary deafness in two unrelated families. Genetically engineered mice homozygous for one of the variants are profoundly deaf, whereas heterozygous mice have normal hearing ...
Evan B. Hale, Barbara Vona, Richard J. Goodyear, Richard T. Osgood, Jun Shen, Sami S. Amr, Karen Mojica, Michaela A. H. Hofrichter, Jörg Schröder, Sophie Flandin, Paul Gratias, Ricardo Vera‐Monroy, Katherine Callahan, Kerry L. Gudlewski, Rolen Quadros, Masato Ohtsuka, JoAnn McGee, Edward J. Walsh, Cynthia C. Morton, Thomas Haaf, Channabasavaiah Gurumurthy, James E. Saunders, Guy P. Richardson, Artur A. Indzhykulian   +23 more
wiley   +1 more source

Early diagnosis of acute renal injury in patients with acute decompensation of chronic heart failure [PDF]

Терапевтический архив, 2019
Early diagnosis of acute kidney injury (AKI) is an urgent problem of providing medical care to patients with acute decompensation of chronic heart failure (ADHF). Aim.
S N Nasonova, I V Zhirov, M V Ledyakhova, T V Sharf, E G Bosykh, V P Masenko, S N Tereshchenko   +6 more
doaj   +1 more source

Preoperative FIB‐4 Index as a Potential Factor Associated With Severe Postoperative Complications and Endogenous Organ Failure After Hepatectomy for Hepatocellular Carcinoma

Annals of Gastroenterological Surgery, EarlyView.
This study demonstrates that a preoperative FIB‐4 index ≥ 5.0 independently predicts severe complications and endogenous organ failure (EOF) following hepatectomy for hepatocellular carcinoma. By capturing structural liver fragility and systemic vulnerability, the FIB‐4 index enhances surgical risk stratification beyond traditional functional markers ...
Masanori Nakamura, Yukiyasu Okamura, Osamu Aramaki, Nao Yoshida, Yusuke Mitsuka, Hayato Abe, Shunsuke Yamagishi, Yasuhiro Okumura, Kyongsun Pak, Shinobu Masuda   +9 more
wiley   +1 more source

A Case of Multiple Mitochondrial Dysfunctions Syndrome 1 and Review of the Literature

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Multiple mitochondrial dysfunctions syndrome 1 (MMDS1, MIM #605711) due to NFU1 gene defects is an ultra‐rare autosomal recessive inborn error of metabolism associated with reduced function of NFU1 iron–sulfur cluster (ISC) scaffold protein.
Charles R. DiFalco, Aaron Williams, Claudia Soler‐Alfonso, Emily Waskow, Elizabeth Mizerik, Fernando Scaglia, Chaya N. Murali   +6 more
wiley   +1 more source