TIMM50 抗体 (AA 8-324)
Our Local Distributor
北京 101111
Quick Overview for TIMM50 抗体 (AA 8-324) (ABIN7876820)
抗原
See all TIMM50 抗体适用
宿主
克隆类型
标记
应用范围
-
-
抗原表位
- AA 8-324
-
原理
- TIMM50 Antibody / Translocase of inner mitochondrial membrane 50
-
纯化方法
- Immunogen affinity purified
-
免疫原
- E.coli-derived human TIMM50 recombinant protein (Position: F8-Q324) was used as the immunogen for the TIMM50 antibody.
-
亚型
- IgG
-
-
anti-Translocase of Inner Mitochondrial Membrane 50 Homolog (TIMM50) antibody
TIMM50 适用: 人 WB, IHC, IF 宿主: 兔 Monoclonal unconjugated
anti-Translocase of Inner Mitochondrial Membrane 50 Homolog (TIMM50) antibodyKD Validated TIMM50 适用: 人 WB, FACS 宿主: 兔 Monoclonal 24GB2670 unconjugated Recombinant Antibody
anti-Translocase of Inner Mitochondrial Membrane 50 Homolog (TIMM50) antibodyKD Validated TIMM50 适用: 人 WB, FACS 宿主: 兔 Monoclonal 24GB5300 unconjugated Recombinant Antibody
anti-Translocase of Inner Mitochondrial Membrane 50 Homolog (TIMM50) (AA 87-353) antibodyTIMM50 适用: 人 WB, ELISA, IHC 宿主: 兔 Polyclonal unconjugated
anti-Translocase of Inner Mitochondrial Membrane 50 Homolog (TIMM50) (AA 396-409) antibodyVerified TIMM50 适用: 人 WB, ELISA 宿主: 山羊 Polyclonal unconjugated
anti-Translocase of Inner Mitochondrial Membrane 50 Homolog (TIMM50) (AA 301-400) antibodyTIMM50 适用: 人 WB, ELISA 宿主: 小鼠 Polyclonal unconjugated
anti-Translocase of Inner Mitochondrial Membrane 50 Homolog (TIMM50) (AA 74-104), (N-Term) antibodyTIMM50 适用: 人 WB 宿主: 兔 Polyclonal RB39839 unconjugated
-
-
应用备注
- Optimal dilution of the TIMM50 antibody should be determined by the researcher.
-
限制
- 仅限研究用
-
-
-
状态
- Lyophilized
-
溶解方式
- Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL
-
缓冲液
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
-
储存条件
- 4 °C,-20 °C
-
储存方法
- After reconstitution, the TIMM50 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
-
-
- TIMM50 (Translocase of Inner Mitochondrial Membrane 50 Homolog (TIMM50))
-
别名
- TIMM50
-
背景
-
TIMM50 antibody recognizes Translocase of inner mitochondrial membrane 50, a mitochondrial inner membrane protein encoded by the TIMM50 gene on chromosome 19q13.2. TIMM50 is a key component of the TIM23 complex, which mediates the import of preproteins from the cytosol into the mitochondrial matrix or inner membrane. This protein functions as the receptor subunit of the TIM23 complex, recognizing presequence-containing mitochondrial proteins and guiding them through the translocase channel. TIMM50 antibody identifies a mitochondrial protein critical for energy metabolism, apoptosis regulation, and mitochondrial biogenesis.
Structurally, TIMM50 consists of an N-terminal mitochondrial targeting sequence, a single transmembrane domain anchoring it to the inner membrane, and a large C-terminal domain extending into the mitochondrial matrix. The matrix-exposed domain interacts directly with imported polypeptides and regulatory subunits such as TIM23 and TIM17. TIMM50 also contains conserved residues required for phosphatase-like activity and structural stabilization of the import pore. Its expression is ubiquitous but highest in energy-demanding tissues such as heart, skeletal muscle, and brain.
TIMM50 functions within the mitochondrial protein import pathway, ensuring proper localization of enzymes required for oxidative phosphorylation (OXPHOS). Disruption of TIMM50 function leads to accumulation of preproteins in the cytosol and mitochondrial dysfunction. The protein participates in maintaining mitochondrial membrane potential and overall organelle integrity. In addition to import regulation, TIMM50 has been linked to apoptosis modulation through interactions with BCL-2 family proteins.
Clinically, biallelic pathogenic variants in TIMM50 cause combined oxidative phosphorylation deficiency 35 (COXPD35), characterized by developmental delay, hypotonia, seizures, and elevated lactate levels. Loss of TIMM50 function impairs respiratory chain complex assembly and ATP production, reflecting its essential role in mitochondrial bioenergetics. Some studies also associate TIMM50 mutations with epilepsy and progressive encephalopathy, highlighting its neurodevelopmental relevance.
Pathway involvement of TIMM50 includes mitochondrial protein import machinery and the oxidative phosphorylation pathway, where it contributes indirectly to ATP generation and ROS regulation. Structural and functional conservation of the TIM23 complex across eukaryotes underscores the evolutionary importance of TIMM50 in mitochondrial function. The protein family classification places TIMM50 within the TIM/TOM translocase family responsible for mitochondrial protein trafficking.
Immunohistochemical staining using TIMM50 antibody reveals strong mitochondrial localization in cardiac and neuronal tissues. The TIMM50 antibody from NSJ Bioreagents provides a robust tool for research into mitochondrial import mechanisms, bioenergetics, and inherited mitochondrial disease. -
UniProt
- Q3ZCQ8
抗原
-
