人 IKBKG 抗体对
01AB01727
Inhibitor of nuclear factor kappa B kinase regulatory subunit gamma (IKBKG), also known as NEMO (NF-kappa B essential modulator), is the regulatory subunit of the inhibitor of nuclear factor kappa-B kinase (IKK) complex, critical for activating the NF-κB signaling pathway. Structurally, NEMO serves as a scaffold that assembles and activates the IKK complex by bridging catalytic subunits IKKα and IKKβ, leading to phosphorylation and degradation of IκB inhibitors and release of NF-κB transcription factors. This activation controls genes regulating inflammation, immunity, cell survival, and development. Beyond its role in NF-κB signaling, NEMO also promotes autophagosomal clearance of misfolded protein aggregates, contributing to proteostasis. Pathogenic mutations in IKBKG cause incontinentia pigmenti (IP), an X-linked dominant disorder causing skin, hair, teeth, and central nervous system abnormalities, primarily affecting females due to male lethality. Mutations also underlie ectodermal dysplasia with immunodeficiency and progressive neurodegeneration resulting from defective NF-κB signaling and impaired protein quality control.
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技术参数
- 靶点基因名
- IKBKG
- 基因别名
- FIP3; NEMO
- 蛋白别名
- inhibitor of nuclear factor kappa B kinase regulatory subunit gamma; IKBKG
- UniProt
- Q9Y6K9
- 物种
- Human
产品详情
- 靶点名称
- IKBKG
- 蛋白名称
- inhibitor of nuclear factor kappa B kinase regulatory subunit gamma
- 蛋白别名
- NF-kappa-B essential modulator; NEMO; FIP-3; IkB kinase-associated protein 1; IKKAP1; Inhibitor of nuclear factor kappa-B kinase subunit gamma; I-kappa-B kinase subunit gamma; IKK-gamma; IKKG; IkB kinase subunit gamma; NF-kappa-B essential modifier
- 靶点基因名
- IKBKG
- 基因别名
- FIP3; NEMO
- UniProt
- Q9Y6K9
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Inhibitor of nuclear factor kappa B kinase regulatory subunit gamma (IKBKG), also known as NEMO (NF-kappa B essential modulator), is the regulatory subunit of the inhibitor of nuclear factor kappa-B kinase (IKK) complex, critical for activating the NF-κB signaling pathway. Structurally, NEMO serves as a scaffold that assembles and activates the IKK complex by bridging catalytic subunits IKKα and IKKβ, leading to phosphorylation and degradation of IκB inhibitors and release of NF-κB transcription factors. This activation controls genes regulating inflammation, immunity, cell survival, and development. Beyond its role in NF-κB signaling, NEMO also promotes autophagosomal clearance of misfolded protein aggregates, contributing to proteostasis. Pathogenic mutations in IKBKG cause incontinentia pigmenti (IP), an X-linked dominant disorder causing skin, hair, teeth, and central nervous system abnormalities, primarily affecting females due to male lethality. Mutations also underlie ectodermal dysplasia with immunodeficiency and progressive neurodegeneration resulting from defective NF-κB signaling and impaired protein quality control.