CIITA
Sign in to saveAlso known as C2TA, CIITAIV, MHC2TA, NLRA, class II, major histocompatibility complex, transactivator, class II major histocompatibility complex transactivator
CIITA is a human gene which encodes a protein called the class II, major histocompatibility complex, transactivator. Mutations in this gene are responsible for the bare lymphocyte syndrome in which the immune system is severely compromised and cannot effectively fight infection. Chromosomal rearrangement of CIITA is involved in the pathogenesis of Hodgkin lymphoma and primary mediastinal B cell lymphoma.
Gene data
CIITA- Name
- class II major histocompatibility complex transactivator
- Type
- protein-coding
- Aliases
- C2TA, CIITAIV, MHC2D1, MHC2TA, NLRA
This gene encodes a protein with an acidic transcriptional activation domain, 4 LRRs (leucine-rich repeats) and a GTP binding domain. The protein is located in the nucleus and acts as a positive regulator of class II major histocompatibility complex gene transcription, and is referred to as the 'master control factor' for the expression of these genes. The protein also binds GTP and uses GTP binding to facilitate its own transport into the nucleus. Once in the nucleus it does not bind DNA but rather uses an intrinsic acetyltransferase (AT) activity to act in a coactivator-like fashion. Mutations in this gene have been associated with bare lymphocyte syndrome type II (also known as hereditary MHC class II deficiency or HLA class II-deficient combined immunodeficiency), increased susceptibility to rheumatoid arthritis, multiple sclerosis, and possibly myocardial infarction. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Nov 2013].
via MyGene.info
Gene · Ensembl
class II major histocompatibility complex transactivator
- Symbol
- CIITA
- Biotype
- Protein coding
- Organism
- Homo sapiens
- Location
- 16:10,866,222-10,943,021
- Strand
- Forward (+)
- Assembly
- GRCh38
via Ensembl · EMBL-EBI
Research
1,417 papers- The NLR member CIITA: Master controller of adaptive and intrinsic immunity and unexpected tool in cancer immunotherapy.Biomedical journal · 2023
- CIITA-modified glioblastomas vaccinate and induce cross-protection against heterologous wild-type glioblastomas.Journal of translational medicine · 2025
- IL-1β-mediated suppression of CIITA attenuates IFN-γ-induced MHC-II expression on Fibroblasts.Cell communication and signaling : CCS · 2025
- ZBTB48 is a priming factor regulating B-cell-specific CIITA expression.The EMBO journal · 2024
- Osteocyte CIITA aggravates osteolytic bone lesions in myeloma.Nature communications · 2022
via PubMed
Wikidata facts
Show 5 more facts
- HomoloGene ID
- 207
- genomic start
- 10971055
- exact match
- identifiers.org/ncbigene/4261
- genomic end
- 10943021
- cytogenetic location
- 16p13.13
via Wikidata · CC0
~2 min read
Article
5 sectionsContents
- Function
- Interactions
- See also
- References
- External links
CIITA is a human gene which encodes a protein called the class II, major histocompatibility complex, transactivator. Mutations in this gene are responsible for the bare lymphocyte syndrome in which the immune system is severely compromised and cannot effectively fight infection. Chromosomal rearrangement of CIITA is involved in the pathogenesis of Hodgkin lymphoma and primary mediastinal B cell lymphoma.
== Function == CIITA mRNA can only be detected in human leukocyte antigen (HLA) system class II-positive cell lines and tissues. This highly restricted tissue distribution suggests that expression of HLA class II genes is to a large extent under the control of CIITA. However, CIITA does not appear to directly bind to DNA. Instead CIITA functions through activation of the transcription factor RFX5. Hence CIITA is classified as a transcriptional coactivator.