Ask about this productRelated genes to: KLHL11 antibody
- Gene:
- KLHL11 NIH gene
- Name:
- kelch like family member 11
- Previous symbol:
- -
- Synonyms:
- FLJ10572
- Chromosome:
- 17q21.2
- Locus Type:
- gene with protein product
- Date approved:
- 2002-08-16
- Date modifiied:
- 2016-10-05
Related products to: KLHL11 antibody
Related articles to: KLHL11 antibody
- Anti-Kelch-like protein 11 (KLHL11) antibody encephalitis is a rare autoimmune neurological disorder, typically characterized by cerebellar syndrome and brainstem involvement, with magnetic resonance imaging (MRI) lesions predominantly localized to the brainstem and cerebellum consistent with rhombencephalitis. Epilepsy as the primary clinical presentation of this disease has been rarely reported. Herein, we describe a case of anti-KLHL11 encephalitis in which the patient presented with recurrent staring spells as the chief complaint, accompanied by transient mild cerebella signs. Brain imaging and routine cerebrospinal fluid (CSF) tests were unremarkable during the first two admissions; only generalized slow waves were detected on electroencephalogram (EEG) in the second admission. At the third admission, brain MRI revealed T2 hyperintensities in the left frontal and parietal lobes and CSF examination showed elevated protein levels; subsequent antibody testing confirmed anti-KLHL11-IgG positivity, thereby establishing a definitive diagnosis. The patient had negative results on tumor screening and exhibited a favorable response to therapy with corticosteroids, intravenous immunoglobulin and efgartigimod. This case highlights the diverse clinical manifestations and staged progression of anti-KLHL11 encephalitis, which warrants full recognition in clinical practice. For patients suspected of autoimmune encephalitis, dynamic re-examination of cranial imaging and CSF, as well as timely KLHL11-IgG testing, are critical for early diagnosis and prompt treatment, thereby improving prognosis. Additionally, this case expands the current understanding of anti-KLHL11 antibody encephalitis regarding its clinical presentations, imaging features, and therapeutic responsiveness. - Source: PubMed
Publication date: 2026/04/10
Xie YingyuChen JunlingXu WeifanZhu YongdongZhou HoushiCai Tongtong - KLHL11 encephalitis is often associated with vestibular symptoms and ataxia. However, the specific disorders of the vestibular and oculomotor systems have only been partially investigated so far. We present a case of paraneoplastic KLHL11 encephalitis in which vestibulo-oculomotor function was quantitatively assessed. - Source: PubMed
Publication date: 2026/04/06
Schwarz Felix KonstantinHöftberger RomanaEndmayr VerenaLukac InesRommer Paulus SRath JakobBerger ThomasMilenkovic IvanWiest Gerald - Germ cell tumors (GCTs) pose significant diagnostic challenges because of the limited performance of existing tumor markers. Here, we used phage immunoprecipitation sequencing (PhIP-Seq) to develop a unique immunosignature panel to improve diagnosing and differentiating GCT. Using 427 serum samples (150 GCT, 277 controls), we developed and validated an immunosignature panel (GCT-iSIGN) comprising 24 peptides from 16 unique proteins. This panel achieved 93% sensitivity, 99% specificity, and an area under the curve (AUC) of 0.98, identifying 23/24 biomarker-negative GCT cases. A secondary model (Sem-iSIGN), consisting of 17 peptides from five proteins, differentiated seminoma from nonseminoma with 96% specificity, 65% sensitivity, and AUC of 0.77. RNA sequencing data from The Cancer Genome Atlas confirmed differential overexpression of target antigens in testicular cancer. ELISA validation of ERVK7 and LUZP4 and immunohistochemical detection of ERVK7, MUC4, ZNF91, and LUZP4 in tumor tissues supported target expression. This study highlights PhIP-Seq immunoprofiling to identify serum-based immunosignature panels that can serve as biomarkers for GCTs. This approach addresses the shortcomings of conventional markers and offers a scalable, cost-effective tool for improving cancer diagnosis and management. - Source: PubMed
Publication date: 2026/04/01
Hammami M BakriKnight Andrew MKherbek HaidaraCostello Brian ADe Lorenzo Silvana BLeibovich Bradley CCheville John CGuo YongLaFrance-Corey Reghann GParamasivan Naveen KHinson Shannon RSagen JessicaOlson Janet EAlgeciras-Schimnich AliciaPittock Sean JMills John RDasari SurendraDubey Divyanshu - Autoimmune etiologies of seizures have been increasingly recognized with the discovery of novel autoantibodies to cell surface and intracellular antigens. GAD65 neurologic autoimmunity has been associated with stiff person syndrome, cerebellar ataxia, and chronic focal epilepsy, usually of temporal regional origin. While musicogenic reflex seizures have been described in multiple GAD65 IgG seropositive patients, non-musicogenic acoustic reflex seizures have not been described. We present a case of a patient with GAD65 antibody associated epilepsy with a long-standing history of right temporal seizures who developed left temporal reflex seizures triggered by a specific character and language while using the language learning app, Duolingo™. - Source: PubMed
Publication date: 2026/01/19
Hass Reece MDubey DivyanshuVan Gompel Jamie JBritton Jeffrey WSmith Kelsey M - In neurological disorders associated with autoantibodies against intracellular antigens, response to therapy tends to be poor and is associated with irreversible neuronal death. This study aimed to analyze autoimmune encephalitis (AE) associated with antibodies (Abs) against intracellular antigens in children, clarify the clinical characteristics of the disease. - Source: PubMed
Publication date: 2026/01/16
Zhang LinlinChe ChaoHan XingyuCao Aihua