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Recombinant Human MIC19 protein is a Human Full Length protein, in the 1 to 277 aa range, expressed in Escherichia coli, with >80% purity and suitable for SDS-PAGE, MS.

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Images

SDS-PAGE - Recombinant Human MIC19 protein (AB171478), expandable thumbnail

Key facts

Purity
>80% SDS-PAGE
Expression system
Escherichia coli
Tags
His tag N-Terminus
Applications
SDS-PAGE, MS
Biologically active
No

Amino acid sequence

M G S S H H H H H H S S G L V P R G S H M G S M G G T T S T R R V T F E A D E N E N I T V V K G I R L S E N V I D R M K E S S P S G S K S Q R Y S G A Y G A S V S D E E L K R R V A E E L A L E Q A K K E S E D Q K R L K Q A K E L D R E R A A A N E Q L T R A I L R E R I C S E E E R A K A K H L A R Q L E E K D R V L K K Q D A F Y K E Q L A R L E E R S S E F Y R V T T E Q Y Q K A A E E V E A K F K R Y E S H P V C A D L Q A K I L Q C Y R E N T H Q T L K C S A L A T Q Y M H C V N H A K Q S M L E K G G

Reactivity data

Application
SDS-PAGE
Reactivity
Reacts
Dilution info
-
Notes

-

Application
MS
Reactivity
Reacts
Dilution info
-
Notes

-

Target data

Function

Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane (PubMed:25781180, PubMed:32567732, PubMed:33130824). Plays an important role in the maintenance of the MICOS complex stability and the mitochondrial cristae morphology (PubMed:25781180, PubMed:32567732, PubMed:33130824). Has also been shown to function as a transcription factor which binds to the BAG1 promoter and represses BAG1 transcription (PubMed:22567091).

Alternative names

Recommended products

Recombinant Human MIC19 protein is a Human Full Length protein, in the 1 to 277 aa range, expressed in Escherichia coli, with >80% purity and suitable for SDS-PAGE, MS.

Key facts

Purity
>80% SDS-PAGE
Expression system
Escherichia coli
Applications
SDS-PAGE, MS
Accession
Q9NX63-1
Animal free
No
Species
Human
Concentration
Loading...
Storage buffer

pH: 8
Constituents: 50% Glycerol (glycerin, glycerine), 1.17% Sodium chloride, 0.32% Tris HCl, 0.03% (R*,R*)-1,4-Dimercaptobutan-2,3-diol

Sequence info

Amino acid sequence

M G S S H H H H H H S S G L V P R G S H M G S M G G T T S T R R V T F E A D E N E N I T V V K G I R L S E N V I D R M K E S S P S G S K S Q R Y S G A Y G A S V S D E E L K R R V A E E L A L E Q A K K E S E D Q K R L K Q A K E L D R E R A A A N E Q L T R A I L R E R I C S E E E R A K A K H L A R Q L E E K D R V L K K Q D A F Y K E Q L A R L E E R S S E F Y R V T T E Q Y Q K A A E E V E A K F K R Y E S H P V C A D L Q A K I L Q C Y R E N T H Q T L K C S A L A T Q Y M H C V N H A K Q S M L E K G G
Accession
Q9NX63
Protein length
Full Length
Predicted molecular weight
28.5 kDa
Amino acids
1 to 277
Nature
Recombinant
Tags
His tag N-Terminus

Specifications

Form
Liquid
Additional notes

ab171478 is purified using conventional chromatography techniques.

General info

Function

Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane (PubMed:25781180, PubMed:32567732, PubMed:33130824). Plays an important role in the maintenance of the MICOS complex stability and the mitochondrial cristae morphology (PubMed:25781180, PubMed:32567732, PubMed:33130824). Has also been shown to function as a transcription factor which binds to the BAG1 promoter and represses BAG1 transcription (PubMed:22567091).

Sequence similarities

Belongs to the MICOS complex subunit Mic19 family. Metazoan Mic19 subfamily.

Subcellular localisation
Mitochondrion inner membrane, Nucleus, Mitochondrion

Storage

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

Notes

This product was previously labelled as CHCHD3

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.
Activity summary

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.

Associated diseases and disorders

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.

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1 product image

  • SDS-PAGE - Recombinant Human MIC19 protein (ab171478), expandable thumbnail

    SDS-PAGE - Recombinant Human MIC19 protein (ab171478)

    15% SDS-PAGE analysis of ab171478 (3µg).

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Product protocols

For this product, it's our understanding that no specific protocols are required. You can:

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