| Field | Specification |
|---|---|
| Mfr No | |
| Clonality | |
| Host | |
| Immunogen | E.coli-derived human TIMM50 recombinant protein (Position: F8-Q324) was used as the immunogen for the TIMM50 antibody. |
| Isotype | |
| Product Type | |
| Purity | |
| Reactivity | |
| Storage | |
| Target | |
| UniProt # |
Overview
TIMM50 Antibody / Translocase of inner mitochondrial membrane 50 is a anti-TIMM50 Rabbit antibody Polyclonal (rabbit origin) supplied in Lyophilized format. Recommended for workflows such as Western blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunofluorescence (IF), Flow cytometry (FACS), ELISA with listed reactivity in Human, Mouse, Rat. Reported localization: Mitochondria, nuclear speckles.
Key elements and design rationale
- Target: TIMM50
- Antibody details: Rabbit, Polyclonal (rabbit origin), isotype Rabbit IgG
- Format: Lyophilized
- Applications (as listed): WB, IHC, ICC/IF, FACS, ELISA
Biological background
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
Research relevance and current trends
- Connecting protein-level changes to phenotype using orthogonal readouts (genetic perturbation, transcriptomics, imaging).
- Considering isoforms and post-translational regulation when interpreting protein-level changes.
- Comparing results across species and model systems with matched controls.
Common research applications
- Western blotting: compare relative abundance and activation-state changes across conditions.
- Immunofluorescence: visualize subcellular distribution and cell-to-cell heterogeneity.
- Immunohistochemistry: map target signal in tissue context and compare regions/phenotypes.
- Flow cytometry: quantify target-positive populations and signal shifts at single-cell resolution.
- ELISA: support antibody-based quantification in assay formats where applicable.
Interpret changes in signal alongside appropriate controls and, when relevant, in parallel with total-protein or pathway readouts.
Notes for experimental interpretation
- Signal can reflect expression level, isoform composition, and post-translational state; interpret results in the context of your model system and stimuli.
- Species differences and sample matrices can influence epitope recognition; prioritize matched controls and orthogonal confirmation when feasible.
Antibody notes: Polyclonal antibodies recognize multiple epitopes, which can broaden the epitope footprint and may increase sensitivity in some contexts.
Customization & Add-ons: Can’t find the antibody you need—or require a custom format for your assay? We can help you source the best match or support custom antibody solutions for diverse research needs, including species and isotype selection, conjugations and labeling (e.g., HRP/AP, biotin, fluorophores), purification grade options (Protein A/G, affinity purified), formulation preferences (buffer selection, carrier-free, glycerol-free), custom concentrations and aliquoting, low-endotoxin options for cell-based work, and application-focused QC/validation support (project dependent). Click Talk to a Scientist to submit a request, email us at support@biohippo.com, or explore our Research Services for additional support—our team will follow up with feasibility details and next steps.
