conjugate
unconjugated
antibody form
purified from hybridoma cell culture
clone
BNP-28, monoclonal
form
liquid
species reactivity
rat, mouse, bovine, human
concentration
~1 mg/mL
technique(s)
immunoblotting: 2-4 μg/mL using whole extracts of human liver cancer cell line HePG2, immunofluorescence: 5-10 μg/mL using human liver cancer cell line HePG2
isotype
IgG2a
UniProt accession no.
shipped in
dry ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
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
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
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
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
Monoclonal Anti-Mitofilin antibody specifically recognizes human and hamster Mitofilin.
Physical form
Supplied as a solution in 0.01 M phosphate buffered saline pH 7.4, containing 15 mM sodium azide as a preservative.
Preparation Note
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.
存储类别
12 - Non Combustible Liquids
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
低风险生物材料
常规特殊物品
此项目有
Walter A Baseler et al.
American journal of physiology. Regulatory, integrative and comparative physiology, 300(2), R186-R200 (2010-11-05)
Diabetic cardiomyopathy is associated with increased risk of heart failure in type 1 diabetic patients. Mitochondrial dysfunction is suggested as an underlying contributor to diabetic cardiomyopathy. Cardiac mitochondria are characterized by subcellular spatial locale, including mitochondria located beneath the sarcolemma
Ralf M Zerbes et al.
Biological chemistry, 393(11), 1247-1261 (2012-10-31)
Mitofilin proteins are crucial organizers of mitochondrial architecture. They are located in the inner mitochondrial membrane and interact with several protein complexes of the outer membrane, thereby generating contact sites between the two membrane systems of mitochondria. Within the inner
Xiaorong Yang et al.
Biophysics reports, 4(2), 104-113 (2018-05-15)
There is accumulating evidence that cysteine sulfenation (cys-SOH) in proteins plays an important role in cellular response to oxidative stress. The purpose of the present study was to identify mitochondrial proteins that undergo changes in cys-SOH during aging. Studies were
Yurong Guo et al.
Molecular & cellular proteomics : MCP, 12(12), 3744-3758 (2013-09-14)
Insulin resistance plays a major role in the development of type 2 diabetes and obesity and affects a number of biological processes such as mitochondrial biogenesis. Though mitochondrial dysfunction has been linked to the development of insulin resistance and pathogenesis
Daniel C Jans et al.
Proceedings of the National Academy of Sciences of the United States of America, 110(22), 8936-8941 (2013-05-17)
The mitochondrial inner membrane organizing system (MINOS) is a conserved large hetero-oligomeric protein complex in the mitochondrial inner membrane, crucial for the maintenance of cristae morphology. MINOS has been suggested to represent the core of an extended protein network that
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