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AFG3L2 Antibody
Rabbit Polyclonal Antibody
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| WB, IHC-P, E |
|---|---|
| Primary Accession | Q9Y4W6 |
| Other Accession | 10939 |
| Reactivity | Human |
| Host | Rabbit |
| Clonality | Polyclonal |
| Isotype | IgG |
| Calculated MW | 88584 Da |
| Dilution | IHC-P (10 µg/ml), |
| Gene ID | 10939 |
|---|---|
| Other Names | AFG3L2, AFG3-like protein 2, SPAX5, Spinocerebellar ataxia 28, Paraplegin-like protein, ATPase family gene 3, yeast, SCA28 |
| Target/Specificity | Human AFG3L2 |
| Reconstitution & Storage | PBS, pH 7.4, 0.03% Proclin 300, 50% glycerol. Aliquot and store at -20°C or -80°C. Avoid freeze-thaw cycles. |
| Precautions | AFG3L2 Antibody is for research use only and not for use in diagnostic or therapeutic procedures. |
| Name | AFG3L2 (HGNC:315) |
|---|---|
| Function | ATP-dependent protease which is essential for axonal and neuron development. In neurons, mediates degradation of SMDT1/EMRE before its assembly with the uniporter complex, limiting the availability of SMDT1/EMRE for MCU assembly and promoting efficient assembly of gatekeeper subunits with MCU (PubMed:27642048). Required for paraplegin (SPG7) maturation (PubMed:30252181). After its cleavage by mitochondrial-processing peptidase (MPP), it converts paraplegin into a proteolytically active mature form (By similarity). Required for the maturation of PINK1 into its 52kDa mature form after its cleavage by mitochondrial-processing peptidase (MPP) (PubMed:22354088, PubMed:30252181). Involved in the regulation of OMA1-dependent processing of OPA1 (PubMed:32600459, PubMed:30252181). Contributes to the proteolytic degradation of GHITM upon hyperpolarization of mitochondria (PubMed:35912435). Progressive GHITM degradation upon persistent hyperpolarization leads to respiratory complex I degradation and broad reshaping of the mitochondrial proteome by AFG3L2 (PubMed:35912435). |
| Cellular Location | Mitochondrion. Mitochondrion inner membrane {ECO:0000250|UniProtKB:Q8JZQ2}; Multi-pass membrane protein |
| Tissue Location | Ubiquitous. Highly expressed in the cerebellar Purkinje cells. |
Thousands of laboratories across the world have published research that depended on the performance of antibodies from Abcepta to advance their research. Check out links to articles that cite our products in major peer-reviewed journals, organized by research category.
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Provided below are standard protocols that you may find useful for product applications.
Background
ATP-dependent protease which is essential for axonal development.
References
Banfi S.,et al.Genomics 59:51-58(1999).
Atorino L.,et al.J. Cell Biol. 163:777-787(2003).
Koppen M.,et al.Mol. Cell. Biol. 27:758-767(2007).
Burkard T.R.,et al.BMC Syst. Biol. 5:17-17(2011).
Edener U.,et al.Eur. J. Hum. Genet. 18:965-968(2010).
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