Oncofusion

start / graph / gene-nbn

Genemoderate confidence 3 of 5: One strong study, or several smaller consistent ones.Q35-encode-reanalysis

NBN / nibrin (O60934, 754 aa) - the MRN subunit whose loss causes a leukaemia-predisposition syndrome and yet is almost dispensable in DepMap: mean -0.047 against MRE11's -0.542 in the same complex, the same call and the same release

also called NBS1 · nibrin · p95 · Nijmegen breakage syndrome protein 1

Children born with two broken copies of this gene get a syndrome that includes a very high risk of blood cancer, yet cancer cell lines in the laboratory barely need it. Those two facts sit oddly together and nobody in this graph had written either of them down.

The same thing, for a scientist

**Named in 3 entries and never written, and it is the most paradoxical member of this tranche.** **The clinical fact.** Biallelic NBN mutation causes Nijmegen breakage syndrome - microcephaly, immunodeficiency, chromosomal instability and a **very high incidence of lymphoid malignancy**. So NBN is a human leukaemia-predisposition gene, which is a stronger claim about relevance to this project than anything in the MRE11, BRCA2 or PALB2 entries. **The screen fact, and it points the other way.** Mean Chronos **-0.047** across 1,258 lines with **1.3%** below -0.5 - essentially flat, and **eleven-fold shallower than MRE11 (-0.542, 56.1%) which is a subunit of the same obligate MRN complex, fetched in the same call against the same release.** NBN is nearer to CBX3 (-0.082) and CBX5 (-0.083) than to any DNA-repair gene in this panel. **Both facts are correct and the graph should hold them together rather than choose.** The plausible reconciliations are that the NBS mutation is hypomorphic rather than null (the common Slavic founder allele produces a truncated protein with residual function), that MRN's fitness requirement is carried by MRE11's nuclease rather than by NBN's scaffolding and ATM-recruiting role, or that the predisposition phenotype is developmental and immunological rather than cell-autonomous fitness. **This beat does not distinguish them** and records the asymmetry as an open question rather than resolving it. **In this disease.** `NBN[tiab] AND KMT2A[tiab]` returns **2**, PMIDs 41669101 and 30478448 - the latter is ribavirin and EIF4E signalling in infant ALL, in which NBN appears without a KMT2A-locus measurement. `nibrin[tiab] AND leukemia[tiab]` returns **7**, all NBS-predisposition epidemiology and none a KMT2A-locus or fusion experiment. No released ENCODE ChIP-seq experiment exists for NBN.

What it looks like
Nibrin754 aa
FHAFHA · residues 24–83BRCT 1BRCT 1 · residues 105–181BRCT 2BRCT 2 · residues 224–3150200400600

No three-dimensional model is available for this protein, so the map above is the whole picture. That is itself informative: AlphaFold does not model chains this long, and the parts that have been crystallised are held under the entries for the individual domains.

Facts and figures
gene symbolNBN
uniprotO60934
uniprot entry nameNBN_HUMAN
gene primary readbackNBN
length AA754
protein nameNibrin
how the accession was resolvedgene_exact:NBN AND organism_id:9606 AND reviewed:true returned exactly ONE reviewed record with primary gene name NBN (rules 62/65). Note that the literature overwhelmingly uses the RETIRED symbol NBS1 and the protein name nibrin, so a search keyed to the current symbol under-recovers - both were run.
domains sourceUniProt O60934 feature table, fetched 2026-09-22
localizationNucleus; Chromosome; Nucleus, PML body; Chromosome, telomere (UniProt O60934)
subnuclear localizationionising-radiation-induced foci; PML nuclear bodies; telomeres
localization shown for fusionNO - nobody has looked in any fusion; FOUR ENCODE TF ChIP-seq experiments DO exist (ENCSR278SQL GM12878, ENCSR210ZYL HepG2, ENCSR591EBL MCF-7, ENCSR085QEV K562) and none has been read at this locus - corrected 2026-09-23, so this is measured-not-analysed rather than never-measured
shown for fusionNO - nobody has looked in any KMT2A fusion
cell typethe predisposition phenotype is lymphoid, which is the closest lineage match of any gene in this tranche; no cell-type-resolved measurement at this locus exists
cell statedamage-conditional
epigenetic readoutnone directly; NBN recruits ATM to breaks, upstream of the H2AX/53BP1 chromatin response but downstream of the H3K9 state this graph's cascade describes
encode statusCORRECTED 2026-09-23, verified live on both ENCODE endpoints by the king: /targets/NBN-human/ returns HTTP 200 and a released-experiment search returns **4 TF ChIP-seq experiments** - ENCSR278SQL (GM12878), ENCSR210ZYL (HepG2), ENCSR591EBL (MCF-7) and ENCSR085QEV (K562). This field previously read 'NO released human TF ChIP-seq experiment for NBN'. The cycle-65 archive sweep reported the four experiments and they were corrected in sibling entries; this one was missed for three cycles, and the reason is structural - see absence_class_note.
absence classnever-measured
relationship to existing entriesgene-mre11 is written in this same tranche and is NBN's partner in the MRN complex; the DepMap asymmetry between them is recorded in both entries so neither can be quoted without the other.
absence class noteTHE CLASS ON THIS ENTRY IS A SUMMARY LABEL AND IT SHOULD NOT BE TRUSTED AS A SINGLE VERDICT. `absence_class` here reads 'never-measured', but this entry carries at least seven distinct absence-bearing fields and they do not share a class: depmap - MEASURED. 1,258 screens, mean gene effect -0.0471. Not an absence at all. encode_status - was FALSE ('no released experiment'); four exist. measured-not-analysed. localization_shown_for_fusion - same false ENCODE clause; corrected the same day. epigenetic_readout - a SCOPING statement ('none directly'), not an absence. cell_type - a genuine absence, no cell-type-resolved measurement. shown_for_fusion - a genuine literature-bounded absence. bounded_absence_2026_09_22 - properly bounded, querytranslations read back. One token cannot carry that, and the false ENCODE clause survived three cycles inside it because a reader checking 'the absence class' saw one plausible word rather than seven claims. Recorded rather than resolved: the single-valued field is the defect, and the fix is a schema change, not a better token.
domains
FHA24.0FHA: 24BRCT 1105.0BRCT 1: 105BRCT 2224.0BRCT 2: 224
gene synonyms in uniprot
[
  "NBS",
  "NBS1",
  "P95"
]
domains — the numbers behind the chart
[
  {
    "name": "FHA",
    "start": 24,
    "end": 83
  },
  {
    "name": "BRCT 1",
    "start": 105,
    "end": 181
  },
  {
    "name": "BRCT 2",
    "start": 224,
    "end": 315
  }
]
depmap
{
  "source": "Open Targets Platform 26.06 (meta.dataVersion year 26 month 06), fetched 2026-09-22",
  "gene_effect_metric": "Chronos",
  "n_screens_this_gene": 1258,
  "mean_gene_effect": -0.0471,
  "n_lines_below_minus_0_5": 16,
  "fraction_below_minus_0_5": 0.0127,
  "min": -0.7939,
  "max": 0.7987,
  "approvedSymbol_readback": "NBN",
  "release_note": "rule 83: no release id in the payload, so the Open Targets dataVersion is recorded. Screen count is gene-specific (1258).",
  "no_median_split": "rule 84: no anti-selective-half tally reported.",
  "live_low_screen_control": "OR4F5 (ENSG00000186092), same session: approvedSymbol reads back but only 3 tissue blocks / 5 screens are returned - a passing symbol readback is not evidence the payload is populated."
}
the paradox
{
  "clinical": "biallelic NBN mutation causes Nijmegen breakage syndrome with a very high incidence of lymphoid malignancy",
  "screen": "mean Chronos -0.047, 1.3% of 1,258 lines below -0.5 - flat",
  "same_complex_comparator": "MRE11 -0.542, 56.1% of 1,258 lines, same call, same release",
  "candidate_reconciliations": [
    "the common NBS founder allele is hypomorphic, not null, so the human phenotype is not a knockout phenotype",
    "MRN's fitness requirement is carried by the MRE11 nuclease and not by the NBN scaffold",
    "the predisposition is developmental and immunological rather than a cell-autonomous fitness defect in an established cancer line"
  ],
  "not_resolved_here": true
}
bounded absence 2026 09 22
{
  "instrument": "PubMed esearch, one [tiab] tag per term, every querytranslation read back",
  "NBN[tiab] AND KMT2A[tiab]": {
    "count": 2,
    "pmids": [
      "41669101",
      "30478448"
    ],
    "on_topic_for_a_kmt2a_locus_measurement": 0
  },
  "nibrin[tiab] AND leukemia[tiab]": {
    "count": 7,
    "querytranslation": "\"nibrin\"[Title/Abstract] AND \"leukemia\"[Title/Abstract]",
    "character": "Nijmegen breakage syndrome predisposition epidemiology",
    "on_topic_for_a_kmt2a_locus_measurement": 0
  },
  "name_list_searched": [
    "NBN",
    "nibrin"
  ],
  "names_NOT_searched": [
    "NBS1 (the retired symbol, and the one most of this literature uses)",
    "p95",
    "'MRN complex' as a phrase"
  ],
  "why_that_matters": "rule 40 - searching only the current HGNC symbol under-counts by up to tenfold when the field publishes under a retired one, and NBS1 is exactly that case. Rule 64 - the complex form was not run either. This absence is bounded by a name list that is known to be incomplete and should be re-run before anyone builds on it.",
  "absence_class": "never-measured"
}
Still open
  1. Which reconciliation of the NBN paradox is right - hypomorphic allele, MRE11-carried fitness, or a developmental rather than cell-autonomous mechanism? RAD50's Chronos profile would discriminate the second and was not fetched.
  2. Is the lymphoid malignancy of Nijmegen breakage syndrome enriched for 11q23 rearrangement? The registries that would answer it exist and the question has not been asked in those words - the same shape as the open question already standing in gene-brca1 about therapy-related leukaemia.
  3. Does the NBN absence survive a search on the retired symbol NBS1 and on the complex name? This entry's own bounded absence is knowingly incomplete on both counts and should not be quoted as a bound until they are run.
Every source behind this entry (2)
  1. E1established confidence 5 of 5: Multiple independent lines of evidence; treated as settled in the field.paper

    Nibrin is a double-strand-break repair protein mutated in Nijmegen breakage syndrome, an autosomal recessive chromosomal-instability disorder whose phenotype includes marked predisposition to lymphoid malignancy.

    What this evidence cannot show

    The founding disease-gene paper. It establishes the gene-disease link and the repair role; the malignancy-incidence figures quoted in this field come from later registry series that were not fetched here, so this entry states the predisposition qualitatively and quotes no rate.

    Evidence that argues against this (1)
    • Nijmegen breakage syndrome predisposes to lymphoma and lymphoid leukaemia broadly; nothing in this literature connects it to KMT2A rearrangement specifically, and a predisposition gene for a lineage is not evidence about a locus. The DepMap profile (-0.047) additionally shows that whatever the mechanism of predisposition is, it is not a cell-autonomous fitness requirement in established lines. Broad Institute DepMap / Open Targets Platform 2026
  2. E2strong confidence 4 of 5: Replicated in peer-reviewed work by more than one group.database

    NBN has a mean DepMap Chronos gene effect of -0.047 across 1,258 lines with 1.3% below -0.5, eleven-fold shallower than MRE11 (-0.542, 56.1%) which is a subunit of the same MRN complex, both fetched in one call against Open Targets dataVersion 26.06.

    DepMap CRISPR gene-effect (Chronos) for NBN, served by the Open Targets Platform GraphQL API · Broad Institute DepMap / Open Targets Platform · Open Targets Platform 26.06 (meta.dataVersion year 26 month 06), fetched 2026-09-22 2026
    What this evidence cannot show

    Rule 83: no DepMap release id in the payload, so the Open Targets dataVersion is recorded; screen counts are gene-specific. Rule 84: no median-split proportion. A flat Chronos score in cancer lines is not evidence that NBN is dispensable in a normal cell, in development, or in the immune system - and the human syndrome shows it is not.

    Evidence that argues against this (1)
    • A near-zero mean gene effect can also mean the screen failed for that gene - poor guide performance, low expression, or a short half-life protein whose knockout is incomplete over the screen window. This entry treats the flat score as a real observation only because it is internally consistent across 1,258 lines and because the live low-screen control (OR4F5, 5 screens) shows the endpoint reports sparse data as sparse rather than as zero. Broad Institute DepMap / Open Targets Platform 2026
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