Human KEOPS subunit / GON7: molecular mechanisms & dependencies

How nutrients influence it

Every nutrient with a recorded effect on this component, credited to the nutrient that acted rather than the chapter that recorded it. Open a nutrient to see the findings and the conditions they were measured under.

Human evidence only

How nutrients reach it in more than one step

Chains of two or more recorded steps that end here, grouped by the nutrient they start from. Each step is a separate finding, so a chain is a route a mechanism could take, not proof that it does.

Tracing routes…

What it does

Every recorded relationship this component is part of, grouped by its role. Plain wording comes first; the technical statement follows.

Recorded relationships

What it acts on

Human GON7 became partly structured upon binding LAGE3 in the GON7/LAGE3/OSGEP subcomplex, supporting its proposed role in KEOPS stability and organization.
Human KEOPS subunit / GON7 → Human KEOPS subunit / LAGE3 source_derived_draftungraded
Experimental context and source evidence
evidence_access
Primary abstract
experimental_model
Crystal structure and cellular characterization of human KEOPS components.
limitations
The structural observation supports a stabilizing role; it does not establish that extra threonine repairs a subunit defect.
nutrient_topic
L-Threonine collection; species, compartment, exposure, co-substrates and manipulation remain explicit. · L-Threonine
plain_language
An organizing subunit helps assemble the RNA-modifying machine.
primary_references
Defects in t6A tRNA modification due to GON7 and YRDC mutations lead to Galloway-Mowat syndrome. · 2019 · https://pubmed.ncbi.nlm.nih.gov/31481669/ · DOI 10.1038/s41467-019-11951-x
L-Threonine: translation, intestinal barrier, metabolism and cross-nutrient mechanisms (2026-09-19) · lines 450–456
AI-assisted research curation; primary references, access levels and experimental limitations individually identified. Not publisher full text. · supports · Crystal structure and cellular characterization of human KEOPS components. · source_derived_draft · unverified_draft
## l-threonine-gon7-complex An organizing subunit helps assemble the RNA-modifying machine. Human GON7 became partly structured upon binding LAGE3 in the GON7/LAGE3/OSGEP subcomplex, supporting its proposed role in KEOPS stability and organization. Model: Crystal structure and cellular characterization of human KEOPS components. Limitations: The structural observation supports a stabilizing role; it does not establish that extra threonine repairs a subunit defect. Evidence access: Primary abstract Defects in t6A tRNA modification due to GON7 and YRDC mutations lead to Galloway-Mowat syndrome. · 2019 · https://pubmed.ncbi.nlm.nih.gov/31481669/ · DOI 10.1038/s41467-019-11951-x
Complete structured claim and evidence

Where it participates (unsigned role)

Inherited YRDC mutations were associated with severe Galloway–Mowat syndrome, linking t6A machinery impairment with microcephaly and early-onset nephrotic disease.
Human threonylcarbamoyladenylate synthase / YRDC → YRDC-associated Galloway–Mowat syndrome source_derived_draftungraded
Experimental context and source evidence
availability_state
machinery_impairment Imported condition classification; unverified.
evidence_access
Primary abstract
experimental_model
Human genetic disease study with functional analyses; GON7-associated cases were milder in the reported series.
limitations
This is an inherited machinery disorder, not demonstrated dietary threonine deficiency or a proven supplementation-responsive syndrome.
nutrient_topic
L-Threonine collection; species, compartment, exposure, co-substrates and manipulation remain explicit. · L-Threonine
plain_language
A threonine-using RNA pathway matters in human brain and kidney development.
primary_references
Defects in t6A tRNA modification due to GON7 and YRDC mutations lead to Galloway-Mowat syndrome. · 2019 · https://pubmed.ncbi.nlm.nih.gov/31481669/ · DOI 10.1038/s41467-019-11951-x
trigger_kind
machinery_impairment Imported condition classification; unverified.
L-Threonine: translation, intestinal barrier, metabolism and cross-nutrient mechanisms (2026-09-19) · lines 458–464
AI-assisted research curation; primary references, access levels and experimental limitations individually identified. Not publisher full text. · supports · Human genetic disease study with functional analyses; GON7-associated cases were milder in the reported series. · source_derived_draft · unverified_draft
## l-threonine-yrdc-inherited-disease A threonine-using RNA pathway matters in human brain and kidney development. Inherited YRDC mutations were associated with severe Galloway–Mowat syndrome, linking t6A machinery impairment with microcephaly and early-onset nephrotic disease. Model: Human genetic disease study with functional analyses; GON7-associated cases were milder in the reported series. Limitations: This is an inherited machinery disorder, not demonstrated dietary threonine deficiency or a proven supplementation-responsive syndrome. Evidence access: Primary abstract Defects in t6A tRNA modification due to GON7 and YRDC mutations lead to Galloway-Mowat syndrome. · 2019 · https://pubmed.ncbi.nlm.nih.gov/31481669/ · DOI 10.1038/s41467-019-11951-x
Complete structured claim and evidence

The events it takes part in

A mechanism often involves more than two components. These are the full events, with every participant and its role.

Situations it appears in

Low-supply and faulty-machinery situations recorded in the chapters where this component plays a part.

In the sources

Preserved passages that mention this component, quoted exactly. Open one to read it in context.

Open hypotheses

Proposed ideas that involve this component. They are labeled as hypotheses and do not change any recorded statement.

This is a research prototype built from draft material. It is not medical advice, and its statements still await verification against the original studies.

Human KEOPS subunit / GON7