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Loss of FBXO11 establishes a stem cell program in acute myeloid leukemia by dysregulating LONP1.

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The Journal of clinical investigation 📖 저널 OA 98.4% 2021: 1/1 OA 2023: 1/1 OA 2024: 1/1 OA 2025: 51/51 OA 2026: 65/67 OA 2021~2026 2026 Vol.136(2) OA
Retraction 확인
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PICO 자동 추출 (휴리스틱, conf 2/4)

유사 논문
P · Population 대상 환자/모집단
환자: AML, which revealed mutations in ubiquitin ligase family genes in 11
I · Intervention 중재 / 시술
추출되지 않음
C · Comparison 대조 / 비교
추출되지 않음
O · Outcome 결과 / 결론
We found that FBXO11 interacts with and catalyzes K63-linked ubiquitination of LONP1 in the cytosol, to promote LONP1 entry into mitochondria.

Kincross H, Mo YA, Wang X, Chang L, Duns G, Mey F

📝 환자 설명용 한 줄

Acute myeloid leukemia (AML) is an aggressive cancer with very poor outcomes.

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↓ .bib ↓ .ris
APA Kincross H, Mo YA, et al. (2026). Loss of FBXO11 establishes a stem cell program in acute myeloid leukemia by dysregulating LONP1.. The Journal of clinical investigation, 136(2). https://doi.org/10.1172/JCI181943
MLA Kincross H, et al.. "Loss of FBXO11 establishes a stem cell program in acute myeloid leukemia by dysregulating LONP1.." The Journal of clinical investigation, vol. 136, no. 2, 2026.
PMID 41289019 ↗
DOI 10.1172/JCI181943

Abstract

Acute myeloid leukemia (AML) is an aggressive cancer with very poor outcomes. To identify additional drivers of leukemogenesis, we analyzed sequencing data from 1,727 unique individual patients with AML, which revealed mutations in ubiquitin ligase family genes in 11.2% of samples from adult patients with AML with mutual exclusivity. The SKP1/CUL1/F-box (SCF) E3 ubiquitin ligase complex gene, FBXO11, was the most significantly downregulated gene of the SCF complex in AML. We found that FBXO11 interacts with and catalyzes K63-linked ubiquitination of LONP1 in the cytosol, to promote LONP1 entry into mitochondria. We show that depletion of FBXO11 or LONP1 reduced mitochondrial respiration through impaired LONP1 chaperone activity to assemble electron transport chain Complex IV. Reduced mitochondrial respiration secondary to FBXO11 or LONP1 depletion imparted myeloid-biased stem cell properties in primary CD34+ hematopoietic stem and progenitor cells (HSPCs) in vitro. In a human xenograft model, depletion of FBXO11 cooperated with AML1-ETO and mutant KRASG12D to generate serially transplantable AML. Our findings suggest that reduced FBXO11 cooperates to initiate AML by priming HSPC for myeloid-biased self renewal through attenuation of LONP1-mediated regulation of mitochondrial respiration.

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