General description
Mitochondria are essential organelles whose function is critical to homeostasis, energy generation, and cell fate.
Their dysfunction leads to various human diseases. Mitochondria membrane is composed of the outer membrane (OMM) and the inner membrane (IMM) where the electron transport chain machinery takes place and the cristae folds of the mitochondria are formed.1 Mitofilin, a transmembrane IMM protein, is a critical core component of the mitochondrial contact site and cristae organizing system (MICOS).Mitofilin is expressed preferentially in the heart3 but also in the brain4, liver5, kidneys6 and eyes7. It is abundantly found at mitochondrial cristae junctions and is also present within cristae.8,9
Specificity
Monoclonal Anti-Mitofilin antibody specifically recognizes human and hamster Mitofilin.
Application
The antibody may be used in various immunochemical techniques including Immunoblotting (~85 kDa) and Immunofluorescence. Detection of the Mitofilin band by Immunoblotting is specifically inhibited by the immunogen.
Biochem/physiol Actions
Mitofilin has critical roles in cristae organization, MICOS formation, ATP generation, protein transport, mitochondrial DNA transcription and apoptosis.1
Mitofilin downregulation or misexpression has been associated with various human diseases such as, diabetes, Parkinson′s and Alzheimer′s diseases, heart failure, hepatic carcinoma, gastric cancer and Glaucoma.1
Physical form
Supplied as a solution in 0.01 M phosphate buffered saline pH 7.4, containing 15 mM sodium azide as a preservative.
Storage and Stability
For continuous use, store at 2-8°C for up to one month. For extended storage, freeze in working aliquots. Repeated freezing and thawing is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilution samples should be discarded if not used within 12 hours.
Disclaimer
Unless otherwise stated in our catalog our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
- UPC:
- 51111753
- Condition:
- New
- Availability:
- 3-5 Days
- Weight:
- 1.00 Ounces
- HazmatClass:
- No
- WeightUOM:
- LB
- MPN:
- SAB4200856-100UL
- Product Size:
- 100/µL