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KLHL6 (Kelch-like protein 6) is a gene whose normal job is to hold cell growth in check. The public catalogues list it as an oncogene driver, a tumour suppressor and a biomarker, and clinical evidence ties its variants to diagnosis, prognosis or drug response. Tied to Non-Hodgkin lymphoma and Diffuse large B-cell lymphoma.
Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin ligase complex that acts as a multifunctional regulator of B-cell receptor (BCR) signalling, germinal centre formation, and T-cell function. Functions by mediating polyubiquitination of target proteins, leading to their proteasomal degradation. In B-cells, plays an essential role in BCR signalling and germinal centre B-cell maturation.
CIViC holds 1 clinical evidence item and 0 assertions across 1 variant. IntOGen calls it a driver in 2 cohorts (1 activating, 1 loss-of-function), covering Malignant Lymphoma, Non-Hodgkin Lymphoma.
In plain words · KLHL6 (Kelch-like protein 6) is a gene whose normal job is to hold cell growth in check. The public catalogues list it as an oncogene driver, a tumour suppressor and a biomarker, and clinical evidence ties its variants to diagnosis, prognosis or drug response. Tied to Non-Hodgkin lymphoma and Diffuse large B-cell lymphoma.
KLHL6 (Kelch-like protein 6) is a gene whose normal job is to hold cell growth in check. The public catalogues list it as an oncogene driver, a tumour suppressor and a biomarker, and clinical evidence ties its variants to diagnosis, prognosis or drug response. Tied to Non-Hodgkin lymphoma and Diffuse large B-cell lymphoma.
Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin ligase complex that acts as a multifunctional regulator of B-cell receptor (BCR) signalling, germinal centre formation, and T-cell function.
No product in this corpus aims at KLHL6 yet. Because the protein is lost rather than overactive, drugs either restore its function or exploit the weakness its loss leaves (synthetic lethality).
Tumour-specific alteration: the catalogues call it an oncogene driver (IntOGen cohort analysis finds it activated more often than chance) and a tumour suppressor (IntOGen finds it knocked out more often than chance), so the direction differs between cohorts but the alteration is somatic either way; what a medicine would aim at or exploit is the altered form or its loss, absent from normal cells; no corpus medicine is aimed at it yet. HPA KLHL6: RNA tissue enriched (lymphoid tissue 29 nTPM); no normal tissue stained high; highest cancer staining lymphoma (3 of 12 high). Distribution: 1 cancer family in the corpus carries a prevalence row, label threshold or catalogue link for it (Lymphoma); Open Targets associates it with 0 specific cancer types at or above 0.5. (Rule 6 of scripts/fetch-target-specificity.ts.)
Sources: UniProt Q8WZ60; CIViC gene KLHL6; IntOGen KLHL6; Human Protein Atlas KLHL6 tissue; Open Targets ENSG00000172578 associations
First described 2003. Earliest sequence paper UniProt cites for the protein: Gupta-Rossi et al, Mol. Immunol, 2003, "Specific over-expression of deltex and a new Kelch-like protein in human germinal center B cells". Source.
Sources: HGNC HGNC:18653 (approved symbol, name, aliases, locus and cross-references (hgnc_complete_set.txt)); UniProt Q8WZ60 (protein name, function text, keywords and locations (REST API)); CIViC gene KLHL6 (1 evidence items, 0 assertions, 1 variants; diseases: Diffuse Large B-cell Lymphoma (GraphQL API, CC0)); IntOGen KLHL6 (driver in 2 cohorts (Act 1, LoF 1); Compendium_Cancer_Genes.tsv release 20240920, CC0 1.0)
Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin ligase complex that acts as a multifunctional regulator of B-cell receptor (BCR) signalling, germinal centre formation, and T-cell function. Functions by mediating polyubiquitination of target proteins, leading to their proteasomal degradation. In B-cells, plays an essential role in BCR signalling and germinal centre B-cell maturation. Targets the BCR signalling subunits CD79A and CD79B for ubiquitin-mediated degradation, thereby controlling surface BCR homeostasis. Mediates polyubiquitination and degradation of the mRNA decay factor roquin-2/RC3H2, which in turn allows accumulation of TNFAIP3, a negative regulator of NF-kappa-B signalling, thereby providing feedback inhibition of BCR-induced NF-kappa-B activation. In T-cells, acts as a dual negative regulator of T-cell exhaustion and mitochondrial dysfunction. Location: Cytoplasm (UniProt). Locus 3q27.1 (HGNC).
RNA: tissue enriched (lymphoid tissue 29 nTPM), detected in some normal tissues.
No normal tissue stained high.
Medium only: carcinoid, cervical cancer, endometrial cancer, glioma.
Human Protein Atlas version 25.1, antibody staining at reliability approved, enhanced or supported; used under CC BY-SA 3.0. Staining counts are patients per level in the atlas cohort, not population prevalence.
Query for this target: (TITLE:"KLHL6" OR ABSTRACT:"KLHL6" OR TITLE:"kelch like family member 6" OR ABSTRACT:"kelch like family member 6" OR TITLE:"Kelch-like protein 6" OR ABSTRACT:"Kelch-like protein 6" OR TITLE:"FLJ00029" OR ABSTRACT:"FLJ00029") AND (cancer OR tumor OR tumour OR oncology OR carcinoma OR lymphoma OR leukemia OR leukaemia OR myeloma OR sarcoma OR melanoma OR glioma). Results are unfiltered search hits about KLHL6, not a curated reading list.