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Mouse Anti-MIEF2 Recombinant Antibody (CBXS-2781) (CBMAB-S5532-CQ)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXS-2781
Application
ELISA, WB

Basic Information

Immunogen
SMCR7 (NP_631901.2, aa1-454) Full length recombinant protein with GST tag
Specificity
Human
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.4
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Mitochondrial Elongation Factor 2
Introduction
This gene encodes an outer mitochondrial membrane protein that functions in the regulation of mitochondrial morphology. It can directly recruit the fission mediator dynamin-related protein 1 (Drp1) to the mitochondrial surface. The gene is located within the Smith-Magenis syndrome region on chromosome 17. Alternative splicing results in multiple transcript variants encoding different isoforms.
Entrez Gene ID
125170
UniProt ID
Q96C03
Alternative Names
Mitochondrial Elongation Factor 2; Smith-Magenis Syndrome Chromosomal Region Candidate Gene 7 Protein; Smith-Magenis Syndrome Chromosome Region, Candidate 7; Mitochondrial Dynamics Protein Of 49 KDa; SMCR7; MID49; Mitochondrial Dynamic Protein Of 49 KDa; Mitochondrial Dynamics Protein MID49; Mitochondrial Dynamic Protein MID49;
Function
Mitochondrial outer membrane protein involved in the regulation of mitochondrial organization (PubMed:29361167).

It is required for mitochondrial fission and promotes the recruitment and association of the fission mediator dynamin-related protein 1 (DNM1L) to the mitochondrial surface independently of the mitochondrial fission FIS1 and MFF proteins. Regulates DNM1L GTPase activity.
Biological Process
Dynamin family protein polymerization involved in mitochondrial fission Source: UniProtKB
Mitochondrion organization Source: UniProtKB
Positive regulation of mitochondrial fission Source: UniProtKB
Positive regulation of protein targeting to membrane Source: UniProtKB
Regulation of mitochondrion organization Source: UniProtKB
Cellular Location
Mitochondrion
Mitochondrion outer membrane
Note: Colocalizes with DNM1L at mitochondrial membrane. Forms foci and rings around mitochondria.
Involvement in disease
Combined oxidative phosphorylation deficiency 49 (COXPD49):
An autosomal recessive, mitochondrial myopathy characterized by progressive muscle weakness, intermittent muscle pain, exercise intolerance, elevated serum creatine kinase, and deficiencies of multiple respiratory chain enzymes.
Topology
Mitochondrial intermembrane: 1-22
Helical: 23-43
Cytoplasmic: 44-454
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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