人 SKIC3 抗体对
01AB02894
SKI3 subunit of superkiller complex (SKIC3) is a cofactor essential for the RNA exosome-mediated 3'–5' mRNA decay. Containing multiple tetratricopeptide repeat (TPR) motifs, SKIC3 serves as a scaffold to position the helicase within the complex, channeling aberrant or ribosome-bound mRNAs to the exosome for degradation. This process is critical for mRNA surveillance and ribosome-associated quality control. Ubiquitously expressed, SKIC3 is vital for cellular homeostasis. Biallelic loss-of-function variants in SKIC3 are the cause of trichohepatoenteric syndrome (THES) type 1, a multisystem disorder characterized by intractable diarrhea, immune dysfunction, and hair abnormalities.
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技术参数
- 靶点基因名
- SKIC3
- 基因别名
- KIAA0372; TTC37
- 蛋白别名
- SKI3 subunit of superkiller complex; SKIC3
- UniProt
- Q6PGP7
- 物种
- Human
产品详情
- 靶点名称
- SKIC3
- 蛋白名称
- SKI3 subunit of superkiller complex
- 蛋白别名
- Superkiller complex protein 3; Ski3; Tetratricopeptide repeat protein 37; TPR repeat protein 37; Tricho-hepatic-enteric syndrome protein; Thespin
- 靶点基因名
- SKIC3
- 基因别名
- KIAA0372; TTC37
- UniProt
- Q6PGP7
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
SKI3 subunit of superkiller complex (SKIC3) is a cofactor essential for the RNA exosome-mediated 3'–5' mRNA decay. Containing multiple tetratricopeptide repeat (TPR) motifs, SKIC3 serves as a scaffold to position the helicase within the complex, channeling aberrant or ribosome-bound mRNAs to the exosome for degradation. This process is critical for mRNA surveillance and ribosome-associated quality control. Ubiquitously expressed, SKIC3 is vital for cellular homeostasis. Biallelic loss-of-function variants in SKIC3 are the cause of trichohepatoenteric syndrome (THES) type 1, a multisystem disorder characterized by intractable diarrhea, immune dysfunction, and hair abnormalities.