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Record W2104441503 · doi:10.1182/blood.v98.4.1003

Defective hematopoiesis and hepatic steatosis in mice with combined deficiencies of the genes encoding Fancc and Cu/Zn superoxide dismutase

2001· article· en· W2104441503 on OpenAlexafffund
Suzana Hadjur, Karen Ung, Louis D. Wadsworth, James E. Dimmick, Evica Rajcan‐Separovic, Richard W. Scott, Manuel Buchwald, Frank R. Jirik

Bibliographic record

VenueBlood · 2001
Typearticle
Languageen
FieldMedicine
TopicHemoglobinopathies and Related Disorders
Canadian institutionsBC Children's HospitalHospital for Sick Children
FundersNational Cancer InstituteCancer Research Society
KeywordsSOD1BiologySuperoxide dismutaseFanconi anemiaHaematopoiesisProgenitor cellBone marrowSOD2Molecular biologyMyeloidImmunologyCancer researchOxidative stressStem cellCell biologyEndocrinologyDNA repairGeneticsGene

Abstract

Several lines of evidence point to an abnormality in the response of Fanconi anemia cells to reactive oxygen species. To investigate the potential pathologic consequences of an in vivo alteration of redox state in mice lacking one of the Fanconi anemia genes, animals were generated having combined deficiencies of the cytosolic Cu/Zn superoxide dismutase (Sod1) and Fanconi anemia complementation group C (Fancc) genes. Interestingly, hepatocytes of Fancc(-/-)Sod1(-/-) mice exhibited a zonal pattern of microvesicular steatosis, possibly as a result of oxidative stress-induced injury to hepatocyte membranes. Consistent with this idea, freshly explanted Fancc(-/-)Sod1(-/-) hepatocytes demonstrated increased spontaneous production of superoxide in vitro. The second phenotypic feature of Fancc(-/-) Sod1(-/-) mice was that of bone marrow hypocellularity accompanied by significant decreases in peripheral blood erythrocyte and leukocyte numbers as compared with wild-type controls. Although flow cytometry analysis with monoclonal antibodies against cell surface antigens revealed normal numbers of primitive hematopoietic progenitor populations in Fancc(-/-)Sod1(-/-) marrow, lineage-positive progenitor numbers were significantly reduced in these mice. Furthermore, the in vitro clonogenic growth of Fancc(-/-)Sod1(-/-) erythroid, myeloid, and early B-lymphoid colonies in semisolid media was profoundly compromised. These results suggested that the altered redox state likely present in Fancc(-/-) Sod1(-/-) hematopoietic progenitors was responsible for an impairment of cell proliferation or survival. (Blood. 2001;98:1003-1011)

Stored with the screening record, where it is evidence for the labels above.

How this classification was reachedexpand

The three-model screen

all 5,600 screened works →

All three models called this out of scope.

stratum: aff_core · design weight: 5595.24 (the sample is stratified; any rate computed without the weight is wrong)
Claude Opus 4.8OUT
genre: empirical
about Canada: no
confidence: high

Mouse genetics of hematopoiesis in Fanconi anemia models.

GPT-5.6 (high)OUT
genre: empirical
about Canada: no
confidence: high

The study examines gene deficiencies and disease phenotypes in mice.

Grok 4.5OUT
genre: empirical
about Canada: no
confidence: high

Mouse genetics of Fanconi anemia and superoxide dismutase; biomedical disease model.

Full frame machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.000
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Bench or experimental · Consensus signal: Bench or experimental
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.002
Threshold uncertainty score0.000

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0010.001
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0020.000
Science and technology studies0.0000.001
Scholarly communication0.0000.000
Open science0.0000.001
Research integrity0.0010.001
Insufficient payload (model declined to judge)0.0020.001

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.007
GPT teacher head0.206
Teacher spread0.199 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designBench or experimental
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations88
Published2001
Admission routes2
Has abstractyes

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