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

