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Mouse Anti-MTX2 Recombinant Antibody (CBFYM-2779) (CBMAB-M2972-FY)

This product is mouse antibody that recognizes MTX2. The antibody CBFYM-2779 can be used for immunoassay techniques such as: WB, IP, IF, ELISA.
See all MTX2 antibodies

Summary

Host Animal
Mouse
Specificity
Mouse, Rat, Human
Clone
CBFYM-2779
Application
WB, IP, IF, ELISA

Basic Information

Specificity
Mouse, Rat, Human
Clonality
Monoclonal
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
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
METAXIN 2
Introduction
The protein encoded by this gene is highly similar to the metaxin 2 protein from mouse, which has been shown to interact with the mitochondrial membrane protein metaxin 1. Because of this similarity, it is thought that the encoded protein is peripherally associated with the cytosolic face of the outer mitochondrial membrane, and that it is involved in the import of proteins into the mitochondrion. Alternative splicing results in multiple transcript variants. A related pseudogene has been identified on chromosome 7.
Entrez Gene ID
Human10651
Mouse53375
Rat288150
UniProt ID
HumanO75431
MouseO88441
RatQ5U1Z9
Alternative Names
Metaxin 2; Mitochondrial Outer Membrane Import Complex Protein 2; Metaxin-2
Function
Involved in transport of proteins into the mitochondrion.
Biological Process
Inner mitochondrial membrane organization Source: UniProtKB
Mitochondrial transport Source: UniProtKB
Mitochondrion organization Source: GO_Central
Protein insertion into mitochondrial outer membrane Source: ComplexPortal
Cellular Location
Mitochondrion outer membrane
Mitochondrion
Involvement in disease
Mandibuloacral dysplasia progeroid syndrome (MDPS):
A form of mandibuloacral dysplasia, a rare progeroid disorder with clinical and genetic heterogeneity, characterized by growth retardation, craniofacial dysmorphic features due to distal bone resorption, musculoskeletal and skin abnormalities associated with lipodystrophy. MDPS is an autosomal recessive disorder. Clinical features include poor growth, osteoporosis, osteopenia, acroosteolysis of distal phalanges, arterial calcification, renal glomerulosclerosis and severe hypertension.

Li, T., Bao, Y., Xia, Y., Meng, H., Zhou, C., Huang, L., ... & Mao, J. (2024). Loss of MTX2 causes mitochondrial dysfunction, podocyte injury, nephrotic proteinuria and glomerulopathy in mice and patients. International Journal of Biological Sciences, 20(3), 937.

Yeter Doğan, B., Günay, N., Ada, Y., & Doğan, M. E. (2023). A novel MTX2 gene splice site variant resulting in exon skipping, causing the recently described mandibuloacral dysplasia progeroid syndrome. American Journal of Medical Genetics Part A, 191(1), 173-182.

Dutta, T., Das, S., Gupta, I., & Koner, A. L. (2022). Construing the metaxin-2 mediated simultaneous localization between mitochondria and nucleolus using molecular viscometry. Chemical Science, 13(44), 12987-12995.

Zhao, Y., Song, E., Wang, W., Hsieh, C. H., Wang, X., Feng, W., ... & Shen, K. (2021). Metaxins are core components of mitochondrial transport adaptor complexes. Nature communications, 12(1), 83.

Jo, Y., Kim, B., & Shin, D. Y. (2021). AREL1 E3 ubiquitin ligase inhibits TNF‑induced necroptosis via the ubiquitination of MTX2. Experimental and Therapeutic Medicine, 22(5), 1-6.

Elouej, S., Harhouri, K., Le Mao, M., Baujat, G., Nampoothiri, S., Kayserili, H., ... & De Sandre-Giovannoli, A. (2020). Loss of MTX2 causes mandibuloacral dysplasia and links mitochondrial dysfunction to altered nuclear morphology. Nature communications, 11(1), 4589.

Adolph, K. W. (2020). Invertebrate metaxins 1 and 2: widely distributed proteins homologous to vertebrate metaxins implicated in protein import into mitochondria. bioRxiv, 2020-01.

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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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