人 PSMB1 抗体对
01AB03431
Proteasome 20S subunit beta 1 (PSMB1) is a critical non-catalytic component of the 20S core proteasome, a multi-subunit complex responsible for the degradation of misfolded, damaged, or regulatory proteins. As part of the 26S proteasome, it facilitates ATP- and ubiquitin-dependent proteolysis, which is essential for maintaining cellular protein homeostasis, regulating the cell cycle, and supporting MHC class I antigen presentation. PSMB1 is ubiquitously expressed and exhibits threonine-type endopeptidase activity within the proteasomal ring structure. Clinically, mutations in the PSMB1 gene are directly linked to a specific neurodevelopmental disorder characterized by microcephaly, hypotonia, and absent language development. Furthermore, its role in tissue differentiation is evidenced by its requirement for proper craniofacial, tendon, and muscle development, where its absence leads to significant structural defects. These associations underscore the fundamental importance of PSMB1 in both systemic protein quality control and specialized developmental processes.
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技术参数
- 靶点基因名
- PSMB1
- 基因别名
- PSC5
- 蛋白别名
- proteasome 20S subunit beta 1; PSMB1
- UniProt
- P20618
- 物种
- Human
产品详情
- 靶点名称
- PSMB1
- 蛋白名称
- proteasome 20S subunit beta 1
- 蛋白别名
- Proteasome subunit beta type-1; Macropain subunit C5; Multicatalytic endopeptidase complex subunit C5; Proteasome component C5; Proteasome gamma chain; Proteasome subunit beta-6; beta-6
- 靶点基因名
- PSMB1
- 基因别名
- PSC5
- UniProt
- P20618
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Proteasome 20S subunit beta 1 (PSMB1) is a critical non-catalytic component of the 20S core proteasome, a multi-subunit complex responsible for the degradation of misfolded, damaged, or regulatory proteins. As part of the 26S proteasome, it facilitates ATP- and ubiquitin-dependent proteolysis, which is essential for maintaining cellular protein homeostasis, regulating the cell cycle, and supporting MHC class I antigen presentation. PSMB1 is ubiquitously expressed and exhibits threonine-type endopeptidase activity within the proteasomal ring structure. Clinically, mutations in the PSMB1 gene are directly linked to a specific neurodevelopmental disorder characterized by microcephaly, hypotonia, and absent language development. Furthermore, its role in tissue differentiation is evidenced by its requirement for proper craniofacial, tendon, and muscle development, where its absence leads to significant structural defects. These associations underscore the fundamental importance of PSMB1 in both systemic protein quality control and specialized developmental processes.