Results 91 to 100 of about 5,775 (200)

Intrinsically Mitochondria‐Targeting Nanozyme via Coordination‐Assembly of Natural Quercetin for Cascade Antioxidant Therapy of Cerebral Ischemia‐Reperfusion Injury

open access: yesAdvanced Science, Volume 13, Issue 50, 7 September 2026.
This study uncovers that quercetin naturally targets mitochondria. By coordinating quercetin with Fe3+, we engineer an ultrasmall cascade nanozyme (MCN) with superoxide dismutase‐catalase activities. MCN crosses the damaged blood–brain barrier, scavenges mitochondrial ROS, prevents mitochondrial DNA leakage, and blocks the cGAS‐STING pathway, thereby ...
Wenxuan Zheng   +14 more
wiley   +1 more source

Role of ATP7A in copper homeostasis

open access: yes, 2007
Menkes disease is a fatal genetic copper deficiency. The Menkes protein (ATP7A) was found to remove copper from tissues in mice that expressed the human ATP7A.
Bi-Xia. Ke (13785013)
core  

Co-localization of ATP7A and GOLPH2 in the internal pyramidal layer.

open access: yes, 2013
The slices were from tx mice subjected to PA administration for 3 days, 10days and 14days, respectively. Golgi apparatus were labeled with GOLPH2. ATP7A(red) staining mainly co-located with GOLPH2(green) in the internal pyramidal layer of tx mice during ...
Xun-Hua Li (322415)   +6 more
core   +1 more source

The Menkes and Wilson disease genes counteract in copper toxicosis in Labrador retrievers: a new canine model for copper-metabolism disorders

open access: yesDisease Models & Mechanisms, 2016
The deleterious effects of a disrupted copper metabolism are illustrated by hereditary diseases caused by mutations in the genes coding for the copper transporters ATP7A and ATP7B. Menkes disease, involving ATP7A, is a fatal neurodegenerative disorder of
Hille Fieten   +20 more
doaj   +1 more source

ATP7A mutation with occipital horns and distal motor neuropathy: A continuum

open access: yesEuropean Journal of Medical Genetics, 2020
ATP7A-related copper transport disorders are classically separated in three pathologies according to their severity, all inherited in an X-linked recessive manner: Menkes disease (MD, OMIM #309400) which represent more than 90% of cases; occipital Horn Syndrome (OHS, OMIM #304150) and ATP7A-related distal motor neuropathy also named X-linked distal ...
Fradin, Melanie   +14 more
openaire   +2 more sources

Solution Structures of the Actuator Domain of ATP7A and ATP7B, the Menkes and Wilson Disease Proteins

open access: yes, 2016
ATP7A and ATP7B are two human P1B-type ATPases that have a crucial role in maintaining copper(I) homeostasis. Among the various domains of these enzymes, one, called the Actuator or A-domain, has a regulatory function and is required for the phosphatase ...
Giovanni Natile (259681)   +6 more
core   +1 more source

Estudo teórico da Interação da Proteína ATP7A com a Cisplatina. [PDF]

open access: yes, 2016
Um quimioterápico muito utilizado no tratamento de diversos tipos de câncer é o cis-diaminodicloroplatina (II), conhecido por cisplatina. Apesar do amplo espectro de atividades apresentado por esse fármaco, fazendo com que ele seja usado para tratar ...
BORGES, Christina Telles
core  

Table_4_Quantitative Ubiquitinomics Revealed Abnormal Ubiquitinated ATP7A Involved in Down-Regulation of ACTH in Silent Corticotroph Adenomas.xls

open access: yes, 2022
Ubiquitination is reported to be a critical biological event on ACTH secretion in corticotroph adenomas. However, the effect of ubiquitylation on ACTH secretion in silent corticotroph adenomas (SCAs) remains unclear.
Hongyun Wang (225359)   +6 more
core   +1 more source

Table_1_Quantitative Ubiquitinomics Revealed Abnormal Ubiquitinated ATP7A Involved in Down-Regulation of ACTH in Silent Corticotroph Adenomas.xls

open access: yes, 2022
Ubiquitination is reported to be a critical biological event on ACTH secretion in corticotroph adenomas. However, the effect of ubiquitylation on ACTH secretion in silent corticotroph adenomas (SCAs) remains unclear.
Hongyun Wang (225359)   +6 more
core   +1 more source

A Truncating De Novo Point Mutation in a Young Infant with Severe Menkes Disease

open access: yesPediatrics and Neonatology, 2017
Menkes disease is a rare neurodegenerative disorder caused by mutations in ATP7A gene. Deficiency in copper-dependent enzymes results in the unique kinky hair appearance, neurodegeneration, developmental delay, seizures, failure to thrive and other ...
Yi-Jie Lin   +8 more
doaj   +1 more source

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