WDR11

Last update 08 May 2025

Basic Info

Synonyms

Bromodomain and WD repeat-containing protein 2, BRWD2, DR11

+ [9]

Introduction

Involved in the Hedgehog (Hh) signaling pathway, is essential for normal ciliogenesis (PubMed:29263200). Regulates the proteolytic processing of GLI3 and cooperates with the transcription factor EMX1 in the induction of downstream Hh pathway gene expression and gonadotropin-releasing hormone production (PubMed:29263200). WDR11 complex facilitates the tethering of Adaptor protein-1 complex (AP-1)-derived vesicles. WDR11 complex acts together with TBC1D23 to facilitate the golgin-mediated capture of vesicles generated using AP-1 (PubMed:29426865).

100 Clinical Results associated with WDR11

100 Translational Medicine associated with WDR11

0 Patents (Medical) associated with WDR11

258

Literatures (Medical) associated with WDR11

01 Oct 2024·The Kaohsiung Journal of Medical Sciences

HLA‐DR genotypes in patients with primary Sjögren's syndrome in Taiwan

Article

Author: Yen, Chang‐Yi ; Tseng, Chia‐Chun ; Ou, Tsan‐Teng ; Wang, Pin‐Yi ; Chen, Kuan‐Yu ; Wu, Cheng‐Chin ; Yen, Jeng‐Hsien

AbstractDifferent human leukocyte antigen (HLA) genotypes have been known to be associated with the risk of development of Sjögren's syndrome in different populations, but this association has never been reported in Taiwan. We enrolled 1044 subjects (673 patients, 371 controls) and tested their HLA‐DR genotypes. We found an increased risk of Sjögren's syndrome in patients carrying HLA‐DR8. DR1 and DR14 were associated with increased risk of eye involvement (uveitis, scleritis or optic neuritis), while DR15 was associated with increased risk of interstitial lung disease. DR8 was associated with increased risk of formation of multiple antibodies: anti‐Ro, rheumatoid factor and antinuclear antibodies (ANA) reaching titer 1:80 or above. DR9 was associated with decreased risk of formation of anti‐La antibodies and increased risk of formation of antithyroglobulin antibodies. DR10 was associated with risk of formation of anticyclic citrullinated peptide (anti‐CCP) antibodies, and DR11 was associated with increased risk of formation of anti‐La antibodies. Oral ulcer was found to be negatively associated with anti‐Ro antibodies and with anti‐ENA antibodies. Skin lesions were associated with ANA antibody titer elevation to 1:80 or above. Malignancies of any kind were associated with the presence of cryoglobulin. Females were more likely to be diagnosed at a younger age than males. There was no statistically significant relationship between HLA‐DR genotype and age at disease diagnosis. In patients with Sjögren's syndrome in Taiwan, the presence of HLA‐DR8 appeared to be a risk factor. In addition, we found several associations between HLA‐DR genotype, clinical presentation, and autoantibody status among them.

01 Aug 2024·Cell

The WDR11 complex is a receptor for acidic-cluster-containing cargo proteins

Article

Author: Wang, Jinrui ; Zhao, Jia ; Tang, Yingying ; Billadeau, Daniel D ; Mo, Xianming ; Zhang, Sitao ; Yong, Xin ; Su, Zhaoming ; Tu, Yingfeng ; Li, Ping ; Jia, Da ; Jia, Guowen ; Deng, Huaqing ; Zhao, Lin ; Yang, Qin

Vesicle trafficking is a fundamental process that allows for the sorting and transport of specific proteins (i.e., "cargoes") to different compartments of eukaryotic cells. Cargo recognition primarily occurs through coats and the associated proteins at the donor membrane. However, it remains unclear whether cargoes can also be selected at other stages of vesicle trafficking to further enhance the fidelity of the process. The WDR11-FAM91A1 complex functions downstream of the clathrin-associated AP-1 complex to facilitate protein transport from endosomes to the TGN. Here, we report the cryo-EM structure of human WDR11-FAM91A1 complex. WDR11 directly and specifically recognizes a subset of acidic clusters, which we term super acidic clusters (SACs). WDR11 complex assembly and its binding to SAC-containing proteins are indispensable for the trafficking of SAC-containing proteins and proper neuronal development in zebrafish. Our studies thus uncover that cargo proteins could be recognized in a sequence-specific manner downstream of a protein coat.

01 May 2024·Diabetes

An Insulin-Chromogranin A Hybrid Peptide Activates DR11-Restricted T Cells in Human Type 1 Diabetes

Article

Author: Callebaut, Aïsha ; Overbergh, Lut ; Hohenstein, Anita C. ; Mathieu, Chantal ; James, Eddie A. ; Gottlieb, Peter A. ; Gallegos, Joylynn B. ; Baker, Rocky L. ; Haskins, Kathryn ; Guyer, Perrin

Hybrid insulin peptides (HIPs) formed through covalent cross-linking of proinsulin fragments to secretory granule peptides are detectable within murine and human islets. The 2.5HIP (C-peptide–chromogranin A [CgA] HIP), recognized by the diabetogenic BDC-2.5 clone, is a major autoantigen in the nonobese diabetic mouse. However, the relevance of this epitope in human disease is currently unclear. A recent study probed T-cell reactivity toward HIPs in patients with type 1 diabetes, documenting responses in one-third of the patients and isolating several HIP-reactive T-cell clones. In this study, we isolated a novel T-cell clone and showed that it responds vigorously to the human equivalent of the 2.5HIP (designated HIP9). Although the responding patient carried the risk-associated DRB1*04:01/DQ8 haplotype, the response was restricted by DRB1*11:03 (DR11). HLA class II tetramer staining revealed higher frequencies of HIP9-reactive T cells in individuals with diabetes than in control participants. Furthermore, in DR11+ participants carrying the DRB4 allele, HIP9-reactive T-cell frequencies were higher than observed frequencies for the immunodominant proinsulin 9-28 epitope. Finally, there was a negative correlation between HIP9-reactive T-cell frequency and age at diagnosis. These results provide direct evidence that this C-peptide–CgA HIP is relevant in human type 1 diabetes and suggest a mechanism by which nonrisk HLA haplotypes may contribute to the development of β-cell autoimmunity.Article Highlights

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WDR11

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