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Mouse Anti-CHCHD2 Recombinant Antibody (CBXC-2764) (CBMAB-C5921-CQ)

This product is a mouse antibody that recognizes CHCHD2. The antibody CBXC-2764 can be used for immunoassay techniques such as: ELISA, IF, IHC, WB.
See all CHCHD2 antibodies

Summary

Host Animal
Mouse
Specificity
Human, Pig, Rat
Clone
CBXC-2764
Antibody Isotype
IgG1
Application
ELISA, IF, IHC, WB

Basic Information

Specificity
Human, Pig, Rat
Antibody Isotype
IgG1
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!]

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
Coiled-Coil-Helix-Coiled-Coil-Helix Domain Containing 2
Introduction
The protein encoded by this gene belongs to a class of eukaryotic CX(9)C proteins characterized by four cysteine residues spaced ten amino acids apart from one another. These residues form disulfide linkages that define a CHCH fold. In response to stress, the protein translocates from the mitochondrial intermembrane space to the nucleus where it binds to a highly conserved 13 nucleotide oxygen responsive element in the promoter of cytochrome oxidase 4I2, a subunit of the terminal enzyme of the electron transport chain. In concert with recombination signal sequence-binding protein J, binding of this protein activates the oxygen responsive element at four percent oxygen. In addition, it has been shown that this protein is a negative regulator of mitochondria-mediated apoptosis. In response to apoptotic stimuli, mitochondrial levels of this protein decrease, allowing BCL2-associated X protein to oligomerize and activate the caspase cascade. Pseudogenes of this gene are found on multiple chromosomes. Alternative splicing results in multiple transcript variants.
Entrez Gene ID
Human51142
Pig100518545
Rat316643
UniProt ID
HumanQ9Y6H1
PigF1RIU9
RatQ5BJB3
Alternative Names
Coiled-Coil-Helix-Coiled-Coil-Helix Domain Containing 2; Aging-Associated Gene 10 Protein; HCV NS2 Trans-Regulated Protein; C7orf17; NS2TP; Coiled-Coil-Helix-Coiled-Coil-Helix Domain-Containing Protein 2, Mitochondrial; Coiled-Coil-Helix-Coiled-Coil-Helix Domain-Containing Protein 2;
Function
Transcription factor. Binds to the oxygen responsive element of COX4I2 and activates its transcription under hypoxia conditions (4% oxygen), as well as normoxia conditions (20% oxygen) (PubMed:23303788).
Biological Process
Cellular response to oxidative stress Source: FlyBase
Mitochondrion organization Source: GO_Central
Positive regulation of mitochondrial ATP synthesis coupled electron transport Source: FlyBase
Positive regulation of transcription by RNA polymerase II Source: UniProtKB
Regulation of cellular response to hypoxia Source: UniProtKB
Cellular Location
Mitochondrion; Mitochondrion intermembrane space; Nucleus. Mainly localised in the intermembrane space.
Involvement in disease
Parkinson disease 22 (PARK22): An autosomal dominant form of Parkinson disease, a complex neurodegenerative disorder characterized by bradykinesia, resting tremor, muscular rigidity and postural instability, as well as by a clinically significant response to treatment with levodopa. The pathology involves the loss of dopaminergic neurons in the substantia nigra and the presence of Lewy bodies (intraneuronal accumulations of aggregated proteins), in surviving neurons in various areas of the brain.

Gundamaraju, R., Lu, W., & Manikam, R. (2021). CHCHD2: The Power House's Potential Prognostic Factor for Cancer?. Frontiers in Cell and Developmental Biology, 8, 1796.

Kee, T. R., Espinoza Gonzalez, P., Wehinger, J. L., Bukhari, M., Ermekbaeva, A., Sista, A., ... & Woo, J. A. A. (2021). Mitochondrial CHCHD2: Disease-Associated Mutations, Physiological Functions, and Current Animal Models. Frontiers in Aging Neuroscience, 13, 209.

Cornelissen, T., Spinazzi, M., Martin, S., Imberechts, D., Vangheluwe, P., Bird, M., ... & Vandenberghe, W. (2020). CHCHD2 harboring Parkinson’s disease-linked T61I mutation precipitates inside mitochondria and induces precipitation of wild-type CHCHD2. Human molecular genetics, 29(7), 1096-1106.

Yao, Y., Su, J., Zhao, L., Li, R., Liu, K., & Wang, S. (2019). CHCHD2 promotes hepatocellular carcinoma and indicates poor prognosis of hepatocellular carcinoma patients. Journal of Cancer, 10(27), 6822.

Mao, C., Wang, H., Luo, H., Zhang, S., Xu, H., Zhang, S., ... & Xu, Y. (2019). CHCHD10 is involved in the development of Parkinson's disease caused by CHCHD2 loss-of-function mutation p. T61I. Neurobiology of aging, 75, 38-41.

Imai, Y., Meng, H., Shiba-Fukushima, K., & Hattori, N. (2019). Twin CHCH proteins, CHCHD2, and CHCHD10: key molecules of Parkinson’s disease, amyotrophic lateral sclerosis, and frontotemporal dementia. International journal of molecular sciences, 20(4), 908.

Zhou, W., Ma, D., Sun, A. X., Tran, H. D., Ma, D. L., Singh, B. K., ... & Tan, E. K. (2019). PD-linked CHCHD2 mutations impair CHCHD10 and MICOS complex leading to mitochondria dysfunction. Human molecular genetics, 28(7), 1100-1116.

Lee, R. G., Sedghi, M., Salari, M., Shearwood, A. M. J., Stentenbach, M., Kariminejad, A., ... & Filipovska, A. (2018). Early-onset Parkinson disease caused by a mutation in CHCHD2 and mitochondrial dysfunction. Neurology Genetics, 4(5).

Tio, M., Wen, R., Lim, Y. L., Zukifli, Z. H. B., Xie, S., Ho, P., ... & Tan, E. K. (2017). Varied pathological and therapeutic response effects associated with CHCHD2 mutant and risk variants. Human mutation, 38(8), 978-987.

Ikeda, A., Matsushima, T., Daida, K., Nakajima, S., Conedera, S., Li, Y., ... & Hattori, N. (2017). A novel mutation of CHCHD2 p. R8H in a sporadic case of Parkinson's disease. Parkinsonism & related disorders, 34, 66-68.

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For research use only. Not intended for any clinical use.

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