Abcam, ab171478, Recombinant Human MIC19 protein

Size: 20µg
Recombinant Human MIC19 protein is a Human Full Length protein, in the 1 to 277 aa range, expressed in Escherichia coli, with >80%, suitable for SDS-PAGE, Mass Spec.
Key facts
Purity:>80% SDS-PAGE,
Expression system:Escherichia coli,
Tags:His tag N-Terminus,
Applications:Mass Spec, SDS-PAGESee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Biologically active:No,
Accession:Q9NX63,
Animal free:No,
Carrier free:No,
Species:Human,
Storage buffer:pH: 8Constituents: 50% Glycerol (glycerin, glycerine), 1.17% Sodium chloride, 0.32% Tris HCl, 0.03% (R*,R*)-1,4-Dimercaptobutan-2,3-diol

Product details:
This product was previously labelled as CHCHD3

Properties and Storage Information:
Shipped at conditions-Blue Ice, Appropriate short-term storage conditions--20°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
MIC19 also known as mitofilin is an integral component of the inner mitochondrial membrane part of the mitochondrial inner membrane organizing system (MINOS). It has a molecular mass of approximately 86 kDa. Expression of MIC19 occurs mainly in tissues with high energy demands including the heart and skeletal muscles. It plays a mechanical role in maintaining the structural integrity of mitochondrial cristae by anchoring specific protein components within the membrane.
Biological function summary
MIC19 contributes to the organization and functional maintenance of mitochondrial architecture by participating in the MINOS complex. This complex regulates the interaction between the inner and outer mitochondrial membranes ensuring efficient mitochondrial dynamics and biogenesis. Furthermore MIC19 supports mitochondrial bioenergetics by stabilizing cristae structures necessary for optimal electron transport chain function.
Pathways
MIC19 integrates into the processes controlling mitochondrial fusion and fission dynamics. It functions within the pathways that are essential for cellular energy metabolism and apoptosis. MIC19 influences these pathways through interactions with proteins such as OPA1 which regulates inner membrane fusion and with components of the electron transport chain. This integrative role highlights MIC19's involvement in maintaining cellular energy homeostasis and programmed cell death.
MIC19 associates with neurodegenerative diseases and cardiomyopathies. Altered expression or mutations in MIC19 can disrupt cristae architecture impairing mitochondrial function and energy production. This disruption relates to the disease processes seen in mitochondrial myopathy where proteins like OPA1 also play a role in mediating mitochondrial dysfunction. The interconnected roles of MIC19 and these associated proteins suggest potential therapeutic targets for intervention in mitochondrial-related conditions.