Component
Human mitochondrial HMG-CoA synthase / HMGCS2
Context-specific entity; species, compartment and exposure are stated on each claim.
2 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.
Where it participates (unsigned role)
Mitochondrial HMGCS2 catalyzes condensation of acetyl-CoA and acetoacetyl-CoA to form HMG-CoA.
Experimental context and source evidence
- evidence_access
- Primary abstract
- experimental_model
- Human HMGCS1/HMGCS2 structures and catalytic reaction description.
- limitations
- The mitochondrial isoform is distinct from cytosolic HMGCS1 in sterol synthesis.
- nutrient_topic
- Fasting physiological-state collection; human protocols, cellular deprivation and refeeding are distinguished. · Fasting / abstention from energy intake
- plain_language
- Two carbon-carrying molecules combine on the ketone-production route.
- primary_references
- Crystal structures of human HMG-CoA synthase isoforms provide insights into inherited ketogenesis disorders and inhibitor design. · 2010 · https://pubmed.ncbi.nlm.nih.gov/20346956/ · DOI 10.1016/j.jmb.2010.03.034
Fasting: fuel switching, nutrient sensing, ketone signaling, nutrient dependencies and refeeding (2026-09-18) · lines 104–110
AI-assisted research curation; primary-abstract references and experimental limitations individually identified. Not publisher full text. · supports · Human HMGCS1/HMGCS2 structures and catalytic reaction description. · source_derived_draft · unverified_draft
## fast-hmgcs2 Two carbon-carrying molecules combine on the ketone-production route. Mitochondrial HMGCS2 catalyzes condensation of acetyl-CoA and acetoacetyl-CoA to form HMG-CoA. Model: Human HMGCS1/HMGCS2 structures and catalytic reaction description. Limitations: The mitochondrial isoform is distinct from cytosolic HMGCS1 in sterol synthesis. Evidence access: Primary abstract Crystal structures of human HMG-CoA synthase isoforms provide insights into inherited ketogenesis disorders and inhibitor design. · 2010 · https://pubmed.ncbi.nlm.nih.gov/20346956/ · DOI 10.1016/j.jmb.2010.03.034
Complete structured claim and evidenceFour patients with HMGCS2 or HMGCL deficiency had decompensation with hypoglycemia and absent urinary ketones.
Experimental context and source evidence
- availability_state
- machinery_impairment Imported condition classification; unverified.
- evidence_access
- Primary abstract
- experimental_model
- Four-patient clinical, biochemical and molecular case series.
- limitations
- Distinct inherited defects; absence of urinary ketones is the reported observation.
- nutrient_topic
- Fasting physiological-state collection; human protocols, cellular deprivation and refeeding are distinguished. · Fasting / abstention from energy intake
- plain_language
- Some bodies cannot make adequate backup fuel under lipolytic stress.
- primary_references
- Inborn Errors of Ketogenesis: Novel Variants, Clinical Presentation, and Follow-Up in a Series of Four Patients. · 2024 · https://pubmed.ncbi.nlm.nih.gov/38567177/ · DOI 10.1055/s-0042-1749362
- trigger_kind
- machinery_impairment Imported condition classification; unverified.
Fasting: fuel switching, nutrient sensing, ketone signaling, nutrient dependencies and refeeding (2026-09-18) · lines 160–166
AI-assisted research curation; primary-abstract references and experimental limitations individually identified. Not publisher full text. · supports · Four-patient clinical, biochemical and molecular case series. · source_derived_draft · unverified_draft
## fast-synthesis-defects Some bodies cannot make adequate backup fuel under lipolytic stress. Four patients with HMGCS2 or HMGCL deficiency had decompensation with hypoglycemia and absent urinary ketones. Model: Four-patient clinical, biochemical and molecular case series. Limitations: Distinct inherited defects; absence of urinary ketones is the reported observation. Evidence access: Primary abstract Inborn Errors of Ketogenesis: Novel Variants, Clinical Presentation, and Follow-Up in a Series of Four Patients. · 2024 · https://pubmed.ncbi.nlm.nih.gov/38567177/ · DOI 10.1055/s-0042-1749362
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.