KLHL11
Kelch-like protein 11
Also known as: FLJ10572, KLH11_HUMAN
Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene
Protein identityUniProt · HPA
- UniProt accession
- Q9NVR0
- Gene
- KLHL11
- Ensembl
- ENSG00000178502
- Chromosome
- 17
- Canonical length
- 708 aa
- Protein class
- Predicted intracellular proteins
- Subcellular location
- Nucleoplasm,Nuclear membrane
- Quaternary structure
- Homodimer
OverviewNCBI Gene
Predicted to enable ubiquitin-like ligase-substrate adaptor activity. Predicted to be involved in proteasome-mediated ubiquitin-dependent protein catabolic process. Predicted to be located in cytosol. Predicted to be part of Cul3-RING ubiquitin ligase complex. Predicted to be active in cytoplasm. [provided by Alliance of Genome Resources, Jul 2025]
Canonical amino-acid sequenceUniProt
708 residues, UniProt reviewed canonical sequence.
>Q9NVR0|KLHL11
1 MAAAAVAAAA AAAAAASLQV LEMESMETAA AGSAGLAAEV RGSGTVDFGP GPGISAMEAS
61 GGDPGPEAED FECSSHCSEL SWRQNEQRRQ GLFCDITLCF GGAGGREFRA HRSVLAAATE
121 YFTPLLSGQF SESRSGRVEM RKWSSEPGPE PDTVEAVIEY MYTGRIRVST GSVHEVLELA
181 DRFLLIRLKE FCGEFLKKKL HLSNCVAIHS LAHMYTLSQL ALKAADMIRR NFHKVIQDEE
241 FYTLPFHLIR DWLSDLEITV DSEEVLFETV LKWVQRNAEE RERYFEELFK LLRLSQMKPT
301 YLTRHVKPER LVANNEVCVK LVADAVERHA LRAENIQSGT CQHPTSHVSL LPRYGQNMDV
361 IMVIGGVSEG GDYLSECVGY FVDEDRWVNL PHIHNHLDGH AVAVTESYVY VAGSMEPGFA
421 KTVERYNPNL NTWEHVCSLM TRKHSFGLTE VKGKLYSIGG HGNFSPGFKD VTVYNPELDK
481 WHNLESAPKI LRDVKALAIE DRFVYIAART PVDRDTEDGL KAVITCYDTE TRQWQDVESL
541 PLIDNYCFFQ MSVVNSNFYQ TASCCPKSYC LENEEAVRKI ASQVSDEILE SLPPEVLSIE
601 GAAICYYKDD VFIIGGWKNS DDIDKQYRKE AYRYCAERKR WMLLPPMPQP RCRATACHVR
661 IPYRYLHGTQ RYPMPQNLMW QKDRIRQMQE IHRHALNMRR VPSSQIECLocalizationUniProt · AlphaFold · HPA
Whether an antibody against KLHL11 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.
- Antibody reachability
- Intracellular
- Secreted
- No
- Transmembrane segments
- 0
- Mean surface accessibility (rSASA)
- 0.3
- Highest tissue expression
- 8.5 nTPM
Expression across tissuesHPA
Tissue
- bone marrow: 8.5 nTPM
- testis: 7.6 nTPM
- retina: 6.5 nTPM
- cerebral cortex: 6.2 nTPM
- parathyroid gland: 4.3 nTPM
- thyroid gland: 4.2 nTPM
Single-cell type
- early spermatids: 158 nCPM
- late spermatids: 59 nCPM
- retinal horizontal cells: 34 nCPM
- rod photoreceptor cells: 27 nCPM
- cone photoreceptor cells: 27 nCPM
- late primary spermatocytes: 26 nCPM
Immune cell
- MAIT T-cell: 0.8 nTPM
- naive B-cell: 0.8 nTPM
- intermediate monocyte: 0.7 nTPM
- naive CD4 T-cell: 0.7 nTPM
- memory B-cell: 0.6 nTPM
- memory CD4 T-cell: 0.6 nTPM
Brain region
- hippocampal formation: 31 nTPM
- cerebellum: 28 nTPM
- cerebral cortex: 27 nTPM
- hypothalamus: 26 nTPM
- basal ganglia: 26 nTPM
- pons: 26 nTPM
DiseaseUniProt · ClinVar · IEDB · PubMed
Four sources answering four different questions about KLHL11.
Disease | AutoantibodyPubMed
Conditions in which antibodies against KLHL11 are reported. Each links to that disease's full target list.
- Encephalitis 15
- Paraneoplastic Syndromes, Nervous System 11
- Autoimmune Diseases of the Nervous System 3
- Cerebellar Ataxia 3
- Hashimoto Disease 3
Showing 5 of 6 — disease pages carrying at least 10 antigens.
ReferencesPubMed · IEDB
Publications for KLHL11 from three distinct lines of evidence, kept separate because they answer different questions: whether antibodies are directed at the protein, whether a B-cell epitope has been mapped on it, and whether a T-cell epitope has. Each is labelled with its source.
Reference: AutoantibodyPubMed
26 publications
- Kelch-like Protein 11 Antibodies in Seminoma-Associated Paraneoplastic Encephalitis.
2019 · N Engl J Med · RCR 9.9 · 219 citations - Expanded Clinical Phenotype, Oncological Associations, and Immunopathologic Insights of Paraneoplastic Kelch-like Protein-11 Encephalitis.
2020 · JAMA Neurol · RCR 7.8 · 141 citations - Clinical significance of Kelch-like protein 11 antibodies.
2020 · Neurol Neuroimmunol Neuroinflamm · RCR 3.9 · 64 citations - Leucine Zipper 4 Autoantibody: A Novel Germ Cell Tumor and Paraneoplastic Biomarker.
2021 · Ann Neurol · RCR 3.7 · 49 citations - Comprehensive Analysis of Paraneoplastic Neurologic Syndrome and PNS-CARE Diagnostic Criteria in Clinical Practice.
2024 · Neurol Neuroimmunol Neuroinflamm · RCR 2.3 · 9 citations
Show 20 more of 26 total
- Pearls & Oy-sters: KLHL11 IgG Paraneoplastic-Associated Hearing Loss and Rhombencephalitis in a Woman With Metastatic Müllerian Tumor.
2024 · Neurology · RCR 1.6 · 7 citations - Kelch-like protein 11 antibody-associated paraneoplastic neurological syndrome: A state-of-the-art review.
2022 · Clin Immunol · RCR 1.6 · 14 citations - Autoimmune movement disorders with neuronal antibodies - an update.
2021 · Curr Opin Neurol · RCR 1.4 · 14 citations - Clinical and serological insights into paraneoplastic brachial amyotrophic diplegia.
2024 · J Neurol · RCR 1.3 · 6 citations - Long-term MRI changes in a patient with Kelch-like protein 11-associated paraneoplastic neurological syndrome.
2021 · Eur J Neurol · RCR 1.2 · 17 citations - Anti-kelchlike protein 11 antibody-associated encephalitis: Two case reports and review of the literature.
2023 · Eur J Neurol · RCR 1.2 · 9 citations - Opsoclonus-myoclonus syndrome associated with anti Kelch-like protein-11 antibodies in a young female patient without cancer.
2021 · J Neuroimmunol · RCR 1.2 · 13 citations - Validation of MATCH score: a predictive tool for identification of patients with kelch-like protein-11 autoantibodies.
2023 · J Neurol Neurosurg Psychiatry · RCR 0.8 · 5 citations - Paraneoplastic Kelch-like protein 11 antibody-associated cerebellar and limbic encephalitis caused by metastatic "burned-out" seminoma - A scar(r)y phenomenon.
2023 · J Neuroimmunol · RCR 0.7 · 7 citations - Clinical Reasoning: A 43-Year-Old Man With Subacute Onset of Vision Disturbances, Jaw Spasms, and Balance and Sleep Difficulties.
2022 · Neurology · RCR 0.5 · 5 citations - Frequency, characteristics, and immunological accompaniments of ataxia in anti-NMDAR antibody-associated encephalitis.
2024 · Front Immunol · RCR 0.4 · 2 citations - KLHL11 antibody-associated autoimmune encephalomyelitis in a middle-aged female patient: a case report and literature review.
2024 · Int J Neurosci · RCR 0.4 · 2 citations - Anti-kelch-like protein 11 antibody-associated encephalitis: two case reports and literature review.
2025 · BMC Neurol · 3 citations - Case Report: KLHL11 encephalitis in a female patient with dual primary malignancies of breast and lung cancer: response to FcRn inhibitor therapy.
2025 · Front Immunol · 3 citations - Anti-Kelch-like protein 11 antibody encephalitis presenting with progressive cognitive deficits in an older woman: a case report and literature review.
2025 · Neurol Sci · 1 citations - Paraneoplastic cerebellitis with Kelch-like protein 11 antibodies as an initial presentation for testicular seminoma.
2025 · BMJ Case Rep - Vestibular and Ocular Motor Phenotype in a Case of Paraneoplastic Autoimmune Kelch-Like Protein-11 Encephalitis.
2026 · Neurol Neuroimmunol Neuroinflamm - Case Report: Atypical anti-KLHL11 antibody encephalitis: recurrent staring spells, serial negative MRI findings, and a tripartite diagnostic journey.
2026 · Front Immunol - Nationwide screening identifies an expanded clinical spectrum of anti-KLHL11 antibody-associated neurologic disease in Japan.
2026 · J Neurol - Clinical Manifestations, Tumor Associations, and Long-Term Outcomes of Anti-KLHL11 Encephalitis.
2026 · Neurol Neuroimmunol Neuroinflamm
Sources: PubMed — antigen-level antibody evidence from a custom retrieval. Records matching a controlled set of autoantibody terms (the MeSH descriptors Autoantibodies and Autoantigens, with title and abstract term variants) were obtained through NCBI E-utilities, and their titles and abstracts parsed for constructions that direct an antibody at a named protein rather than for co-occurrence. Captured names were resolved against UniProt nomenclature and each antigen adjudicated individually against the source text. Bibliographic records from PubMed and MeSH, U.S. National Library of Medicine; citation metrics from NIH iCite (Hutchins et al., PLoS Biology 2016). Titles link to PubMed; abstracts are not reproduced here. The NLM does not endorse this analysis.
Genetic constraint and essentialitygnomAD · DepMap
Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.
- gnomAD LOEUF (loss-of-function intolerance)
- 0.49
- gnomAD pLI
- 0.2
- gnomAD missense Z
- 3.18
- DepMap mean gene effect
- -0.33
- DepMap dependency class
- selective
Cancer expressionTCGA
Across TCGA tumor cohorts, this protein is over-expressed in roughly 4% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).
OntologyGO
Biological processes
Molecular functions
Cellular components
Protein domainsUniProt · Pfam · InterPro
KeywordsUniProt
InteractionsUniProt · HPA
Protein binding partners of KLHL11 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.
Antibody and autoantibody relevanceSeroatlas analysis
Seroatlas reads KLHL11 as an antibody target. Whether an autoantibody or antibody against KLHL11 could matter depends on whether native KLHL11 is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.
KLHL11 is annotated as predominantly intracellular. Intracellular proteins are common autoantibody markers, becoming visible to the immune system after cell injury or altered processing, but are usually markers of disease rather than direct drivers.
Annotation status
The present source text does not explicitly label KLHL11 as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.
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