WB | 1/1000 | Human,Mouse,Rat |
IF | 咨询技术 | Human,Mouse,Rat |
IHC | 1/100-1/500 | Human,Mouse,Rat |
ICC | 技术咨询 | Human,Mouse,Rat |
FCM | 咨询技术 | Human,Mouse,Rat |
Elisa | 咨询技术 | Human,Mouse,Rat |
Aliases | CAAX prenyl protease 1 homolog, Farnesylated proteins-converting enzyme 1, FACE-1, Prenyl protein-specific endoprotease 1, Zinc metalloproteinase Ste24 homolog, ZMPSTE24, FACE1, STE24 |
Entrez GeneID | 10269 |
WB Predicted band size | 54.8kDa |
Host/Isotype | Rabbit IgG |
Antibody Type | Primary antibody |
Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
Species Reactivity | Human, Mouse |
Immunogen | This ZMPSTE24 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 400-430 amino acids from the Central region of human ZMPSTE24. |
Formulation | Purified antibody in PBS with 0.05% sodium azide,1%BSA and 50% glycerol.prepared by Saturated Ammonium Sulfate (SAS) . |
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以下是3篇与ZMPSTE24抗体相关的代表性文献及其摘要概括:
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1. **标题**: *Deficiency of ZMPSTE24 protease in Hutchinson-Gilford progeria syndrome*
**作者**: Bergo MO, et al.
**摘要**: 研究通过Western blot和免疫荧光发现,早衰症患者细胞中ZMPSTE24蛋白表达显著降低,导致前层蛋白A(prelamin A)无法正常加工为成熟层蛋白A,揭示了ZMPSTE24缺失与核结构异常的直接关联。
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2. **标题**: *ZMPSTE24. a metalloprotease linked to progeroid syndromes, is essential for adipocyte differentiation and mitochondrial function*
**作者**: Coffinier C, et al.
**摘要**: 利用ZMPSTE24抗体检测小鼠脂肪组织,发现该蛋白酶通过调控prelamin A加工影响脂肪细胞分化和线粒体功能,提示其代谢调节作用超出早衰症机制。
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3. **标题**: *Nuclear envelope alterations in fibroblasts expressing mutants of ZMPSTE24*
**作者**: Varela I, et al.
**摘要**: 通过免疫组化结合ZMPSTE24抗体,分析突变细胞模型,证实ZMPSTE24功能异常导致核膜结构缺陷,并激活DNA损伤响应通路,为治疗提供潜在靶点。
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**备注**:以上文献为领域内经典研究,实际引用时建议通过PubMed或期刊官网核对完整信息。如需近年研究,可补充关键词(如“ZMPSTE24 antibody 2020-2023”)检索更新成果。
The ZMPSTE24 antibody is a crucial tool in studying the zinc metalloprotease ZMPSTE24. a membrane-bound enzyme essential for processing prelamin A into mature lamin A, a structural component of the nuclear lamina. ZMPSTE24 cleaves the C-terminal farnesylated tail of prelamin A, enabling proper nuclear envelope assembly and function. Mutations in ZMPSTE24 disrupt this process, leading to toxic farnesylated prelamin A accumulation, which is linked to severe genetic disorders like Hutchinson-Gilford progeria syndrome (HGPS) and restrictive dermopathy. These conditions are characterized by premature aging, skeletal abnormalities, and cellular instability.
Researchers use ZMPSTE24 antibodies to detect protein expression levels, localization, and dysfunction in disease models via techniques like Western blotting, immunofluorescence, and immunohistochemistry. Such studies help elucidate ZMPSTE24's role in laminopathies, aging, and genome integrity. Commercially available antibodies are typically raised in rabbits or mice, validated for specificity using knockout controls. Recent work also explores ZMPSTE24's interactions with viral proteins and its potential as a therapeutic target. By enabling precise analysis of ZMPSTE24 dynamics, these antibodies advance our understanding of nuclear biology and pathologies linked to lamin processing defects.
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