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

Name: Mouse Anti-MTX2 Recombinant Antibody (CBFYM-2779)
SKU: CBMAB-M2972-FY
Rating: 5 (1 reviews)

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Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

CBFYM-2779

Application

WB, IP, IF, ELISA

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.

More Infomation

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

Human	10651
Mouse	53375
Rat	288150

UniProt ID

Human	O75431
Mouse	O88441
Rat	Q5U1Z9

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Isotype control

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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