Recombinant TIMM50 抗体
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Quick Overview for Recombinant TIMM50 抗体 (ABIN7882541)
抗原
See all TIMM50 抗体抗体类型
适用
宿主
克隆类型
标记
应用范围
克隆位点
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原理
- TIM50 Antibody / Translocase of inner mitochondrial membrane 50
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纯化方法
- Affinity chromatography
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免疫原
- A synthesized peptide derived from human TIM50 was used as the immunogen for the TIM50 antibody.
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亚型
- IgG
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应用备注
- Optimal dilution of the TIM50 antibody should be determined by the researcher.
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限制
- 仅限研究用
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状态
- Liquid
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缓冲液
- Rabbit IgG in phosphate buffered saline, pH 7.4, 150 mM NaCl, 0.02 % sodium azide and 50 % glycerol, 0.4-0.5 mg/mL BSA
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储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- Store the TIM50 antibody at -20oC.
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- TIMM50 (Translocase of Inner Mitochondrial Membrane 50 Homolog (TIMM50))
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别名
- TIM50
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背景
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TIM50 antibody detects Translocase of inner mitochondrial membrane 50, encoded by the TIMM50 gene. Translocase of inner mitochondrial membrane 50, commonly called TIM50, is an essential component of the TIM23 complex that mediates the import of nuclear encoded mitochondrial proteins. The majority of mitochondrial proteins are encoded in the nucleus, synthesized in the cytoplasm, and imported across the mitochondrial membranes through specialized translocases. TIM50 antibody provides researchers with a critical reagent to study protein import, mitochondrial biogenesis, and energy metabolism.
As a receptor subunit of the TIM23 complex, TIM50 binds to presequences on precursor proteins as they enter the intermembrane space. Research using TIM50 antibody has shown that this interaction is required for recognition and transfer of proteins from the TOM complex in the outer membrane to the TIM23 channel in the inner membrane. TIM50 also acts as a gatekeeper, regulating the opening and closing of the translocation channel in response to membrane potential and precursor signals. This makes TIM50 indispensable for mitochondrial protein import.
Dysfunction of TIMM50 is linked to mitochondrial disease. Mutations in the TIMM50 gene cause combined oxidative phosphorylation deficiency, characterized by developmental delay, seizures, and lactic acidosis. Studies with TIM50 antibody have confirmed that mutations reduce protein import efficiency and disrupt mitochondrial homeostasis. Because proper protein import is essential for assembly of respiratory chain complexes, TIM50 deficiency leads to impaired energy metabolism and neuromuscular symptoms.
Beyond rare genetic syndromes, TIM50 has been implicated in cancer and neurodegeneration. Research with TIM50 antibody has demonstrated altered expression in tumors, where mitochondrial remodeling supports rapid growth. In neurons, reduced TIM50 activity contributes to mitochondrial fragmentation and synaptic dysfunction. These findings highlight its broader significance beyond classical mitochondrial import pathways.
TIM50 antibody is widely used in western blotting, immunohistochemistry, and immunofluorescence. Western blotting detects TIM50 expression in mitochondria rich tissues, immunohistochemistry localizes it to muscle and brain, and immunofluorescence confirms inner membrane localization with colocalization to mitochondrial markers. These applications make TIM50 antibody essential for studying mitochondrial protein transport.
By supplying validated TIM50 antibody reagents, NSJ Bioreagents supports research into protein import, energy metabolism, and disease. Detection of Translocase of inner mitochondrial membrane 50 enables researchers to explore how mitochondrial function is regulated at the level of protein translocation. -
UniProt
- Q3ZCQ8
抗原
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