Component
GSS-deficient tissues from the two sisters studied in 1974
GSS-deficient tissues from the two sisters studied in 1974. Species, exposure and limitations are retained in each linked claim.
1 recorded relationships. Experimental role, claim status and evidence remain attached to each record.
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.
Other things that act on it
Enzymes, hormones, genes, and other components with a recorded effect. These are not nutrients, so they do not count toward the arrows above. Each finding names the chapter that recorded it.
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.
What it acts on
The lesion localized to GSS; findings supported excess gamma-glutamylcysteine-derived 5-oxoproline production beyond disposal capacity.
Experimental context and source evidence
- availability_state
- machinery_impairment Imported condition classification; unverified.
- evidence_span
- {"source_cache": "artifacts/glutathione-research/4152248.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "67120c198b8519f539205ac8148f02ffaf3ae37dc26810a7181cb18887db51d5", "start_char": 0, "end_char": 750, "text_sha256": "67120c198b8519f539205ac8148f02ffaf3ae37dc26810a7181cb18887db51d5"}
- experimental_model
- Enzyme studies in two affected sisters
- exposure
- Inherited 5-oxoprolinuria
- limitations
- Small historical disease study; overproduction mechanism is the authors’ interpretation, not a universal cause of acidosis.
- nutrient_topic
- Glutathione research collection; topical membership is not evidence of a direct dietary effect. · GSH
- organism
- Human
- plain_language
- A blocked assembly pathway can also accumulate a side product.
- primary_references
- [glutathione-p4152248] Glutathione synthetase deficiency, an inborn error of metabolism involving the gamma-glutamyl cycle in patients with 5-oxoprolinuria (pyroglutamic aciduria). (1974). https://pubmed.ncbi.nlm.nih.gov/4152248/ DOI: 10.1073/pnas.71.6.2505
- tissue_or_cell_type
- Placenta, fibroblasts and erythrocytes
- trigger_kind
- machinery_impairment Imported condition classification; unverified.
Glutathione: metabolism, signaling and nutrient connections (2026-09-17) · lines 437–448
AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Enzyme studies in two affected sisters · source_derived_draft · unverified_draft
### glutathione-gss-pyroglutamate The lesion localized to GSS; findings supported excess gamma-glutamylcysteine-derived 5-oxoproline production beyond disposal capacity. Condition category: machinery_impairment nutrient_topic: Glutathione research collection; topical membership is not evidence of a direct dietary effect. plain_language: A blocked assembly pathway can also accumulate a side product. organism: Human tissue_or_cell_type: Placenta, fibroblasts and erythrocytes experimental_model: Enzyme studies in two affected sisters limitations: Small historical disease study; overproduction mechanism is the authors’ interpretation, not a universal cause of acidosis. exposure: Inherited 5-oxoprolinuria evidence_span: {"source_cache": "artifacts/glutathione-research/4152248.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "67120c198b8519f539205ac8148f02ffaf3ae37dc26810a7181cb18887db51d5", "start_char": 0, "end_char": 750, "text_sha256": "67120c198b8519f539205ac8148f02ffaf3ae37dc26810a7181cb18887db51d5"} [glutathione-p4152248] Glutathione synthetase deficiency, an inborn error of metabolism involving the gamma-glutamyl cycle in patients with 5-oxoprolinuria (pyroglutamic aciduria). (1974). https://pubmed.ncbi.nlm.nih.gov/4152248/ DOI: 10.1073/pnas.71.6.2505
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.