ZNF683

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Description

The ZNF683 (zinc finger protein 683) is a protein-coding gene located on chromosome 1.

ZNF683 is a transcription factor that regulates gene expression in various immune cell types, including tissue-resident memory T (Trm) cells, natural killer (trNK) cells, and natural killer T (NKT) cells. It plays a crucial role in the development, retention, and long-term establishment of these T-cell populations in non-lymphoid organs like the skin, gut, liver, and kidney. ZNF683 promotes the development and maintenance of these tissue-resident T-cell populations by suppressing the expression of genes that promote their migration out of these tissues. This function helps ensure a rapid immune response against reactivating infections or viral reinfection. ZNF683 also influences the differentiation of both thymic and peripheral NKT cells, regulating interferon-gamma (IFN-gamma) production and granzyme B production in these cells. It interacts with the transcriptional repressor PRDM1/BLIMP1 at gene promoter regions.

ZNF683 is also known as Hobit.

Associated Diseases

• Miyoshi myopathy
• cancer
• isolated agammaglobulinemia
• combined immunodeficiency with skin granulomas
• agammaglobulinemia 7, autosomal recessive
• common variable immunodeficiency
• immunodeficiency 18
• severe combined immunodeficiency due to IKK2 deficiency
• immunodeficiency, common variable, 4
• immunodeficiency 19
• Okt4 epitope deficiency
• idiopathic CD4 lymphocytopenia
• Cernunnos-XLF deficiency
• T-B+ severe combined immunodeficiency due to IL-7Ralpha deficiency
• severe combined immunodeficiency due to LAT deficiency
• combined immunodeficiency due to partial RAG1 deficiency
• immunodeficiency 73b with defective neutrophil chemotaxis and lymphopenia
• T-cell lymphopenia, infantile, with or without nail dystrophy, autosomal dominant
• combined immunodeficiency due to CD3gamma deficiency
• combined immunodeficiency due to ZAP70 deficiency