AFG3L2 Antibody (N-term)
Affinity Purified Rabbit Polyclonal Antibody (Pab)
- SPECIFICATION
- CITATIONS: 1
- PROTOCOLS
- BACKGROUND
Application
| IHC-P, WB, E |
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Primary Accession | Q9Y4W6 |
Other Accession | Q8JZQ2, Q2KJI7, NP_006787.2 |
Reactivity | Human |
Predicted | Bovine, Mouse |
Host | Rabbit |
Clonality | Polyclonal |
Isotype | Rabbit IgG |
Calculated MW | 88584 Da |
Antigen Region | 52-80 aa |
Gene ID | 10939 |
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Other Names | AFG3-like protein 2, 3424-, Paraplegin-like protein, AFG3L2 |
Target/Specificity | This AFG3L2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 52-80 amino acids from the N-terminal region of human AFG3L2. |
Dilution | WB~~1:1000 IHC-P~~1:10~50 |
Format | Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification. |
Storage | Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles. |
Precautions | AFG3L2 Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures. |
Name | AFG3L2 (HGNC:315) |
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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. |
Provided below are standard protocols that you may find useful for product applications.
Background
This gene encodes a protein localized in mitochondria and closely related to paraplegin. The paraplegin gene is responsible for an autosomal recessive form of hereditary spastic paraplegia. This gene is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders.
References
Edener, U., et al. Eur. J. Hum. Genet. 18(8):965-968(2010)
Di Bella, D., et al. Nat. Genet. 42(4):313-321(2010)
Augustin, S., et al. Mol. Cell 35(5):574-585(2009)
Mariotti, C., et al. Cerebellum 7(2):184-188(2008)
Cagnoli, C., et al. Brain 129 (PT 1), 235-242 (2006) :
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