Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Interaction between the AAA ATPase p97/VCP and a concealed UBX domain in the copper transporter ATP7A is associated with motor neuron degeneration.

The Journal of biological chemistry - 18 May 2018

Yi Ling, Kaler Stephen G

Abstract excerpt

The copper-transporting ATPase ATP7A contains eight transmembrane domains and is required for normal human copper homeostasis. Mutations in the ATP7A gene may lead to infantile-onset cerebral degeneration (Menkes disease); occipital horn syndrome (OHS), a related but much milder illness; or an adult-onset isolated distal motor neuropathy. The ATP7A missense mutation T994I is located in the sixth transmembrane...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Cell Membrane
Share to Copper-Transporting ATPases

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to HEK293 Cells

Share to Motor Neurons

Share to Nerve Degeneration

Share to Protein Domains

Share to Sequence Homology

Share to Valosin Containing Protein

Source

Open in PubMed Open full text

Identifiers

PMID: 29599289
DOI: 10.1074/jbc.RA117.000686
PMCID: PMC5961040

Genes

VCP

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Interaction between the AAA ATPase p97/VCP and a concealed UBX domain in the copper transporter ATP7A is associated with motor neuron degeneration.PMID 29599289Read ↗

Altered intracellular localization and valosin-containing protein (p97 VCP) interaction underlie ATP7A-related distal motor neuropathy.Semantic proximity Read ↗

Direct interactions of adaptor protein complexes 1 and 2 with the copper transporter ATP7A mediate its anterograde and retrograde trafficking.Semantic proximity Read ↗

An overview and update of ATP7A mutations leading to Menkes disease and occipital horn syndrome.Semantic proximity Read ↗

The six metal binding domains in human copper transporter, ATP7B: molecular biophysics and disease-causing mutations.Semantic proximity Read ↗

Mutant p97 exhibits species-specific changes of its ATPase activity and compromises the UBXD9-mediated monomerisation of p97 hexamers.Semantic proximity Read ↗

A glimpse into the regulation of the Wilson disease protein, ATP7B, sheds light on the complexity of mammalian apical trafficking pathways.Semantic proximity Read ↗

Characterizing the molecular phenotype of an Atp7a(T985I) conditional knock in mouse model for X-linked distal hereditary motor neuropathy (dHMNX).Semantic proximity Read ↗

In silico prediction, Characterization, Docking studies and Molecular dynamics simulation of human p97 in complex with p37 cofactorSemantic proximity Read ↗

Copper-transporting ATPases throughout the animal evolution – From clinics to basal neuron-less animalsSemantic proximity Read ↗

The lumenal loop Met672-Pro707 of copper-transporting ATPase ATP7A binds metals and facilitates copper release from the intramembrane sites.Semantic proximity Read ↗

A unique IBMPFD-related P97/VCP mutation with differential binding pattern and subcellular localization.Semantic proximity Read ↗

Dysfunction of ATP7B Splicing Variant Caused by Enhanced Interaction With COMMD1 in Wilson DiseaseSemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.