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Mouse Anti-AFG3L2 Recombinant Antibody (V2-12537) (CBMAB-1027-CN)

This product is a mouse antibody that recognizes AFG3L2 of human. The antibody 2D3 can be used for immunoassay techniques such as: WB.
See all AFG3L2 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
V2-12537
Antibody Isotype
IgG1
Application
WB

Basic Information

Immunogen
Human recombinant protein fragment corresponding to aa. 1-250 of human AFG3L2 (NP_006787) produced in E. coli
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:500

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH7.3, 1% BSA, 50% glycerol
Preservative
0.02% sodium azide
Concentration
1 mg/ml
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
Localized in aa. 1-250

Target

Full Name
AFG3 Like Matrix AAA Peptidase Subunit 2
Introduction
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. ATP-dependent protease which is essential for axonal and neuron development. In neurons, this protein 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.
Entrez Gene ID
10939
UniProt ID
Q9Y4W6
Alternative Names
SCA28; SPAX5
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. Required for the maturation of paraplegin (SPG7) after its cleavage by mitochondrial-processing peptidase (MPP), converting it 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).
Biological Process
Axonogenesis
Calcium import into the mitochondrion
Cristae formation
Membrane protein proteolysis
Mitochondrial calcium ion homeostasis
Mitochondrial calcium ion transmembrane transport
Mitochondrial fusion
Mitochondrial protein processing
Muscle fiber development
Myelination
Nerve development
Neuromuscular junction development
Protein autoprocessing
Protein-containing complex assembly
Protein processing
Proteolysis
Regulation of multicellular organism growth
Righting reflex
Cellular Location
Mitochondrion; Mitochondrion inner membrane
Involvement in disease
Spinocerebellar ataxia 28 (SCA28): Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA28 is an autosomal dominant cerebellar ataxia (ADCA) with a slow progressive course and no evidence of sensory involvement or cognitive impairment.
Spastic ataxia 5, autosomal recessive (SPAX5): A neurodegenerative disorder characterized by early onset spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy.
PTM
Upon import into the mitochondrion, the N-terminal transit peptide is cleaved to generate an intermediate form which undergoes autocatalytic proteolytic processing to generate the proteolytically active mature form.

Caporali, L., Magri, S., Legati, A., Del Dotto, V., Tagliavini, F., Balistreri, F., ... & Taroni, F. (2020). ATPase domain AFG3L2 mutations alter OPA1 processing and cause optic neuropathy. Annals of neurology, 88(1), 18-32.

Puchades, C., Ding, B., Song, A., Wiseman, R. L., Lander, G. C., & Glynn, S. E. (2019). Unique structural features of the mitochondrial AAA+ protease AFG3L2 reveal the molecular basis for activity in health and disease. Molecular cell, 75(5), 1073-1085.

Mancini, C., Hoxha, E., Iommarini, L., Brussino, A., Richter, U., Montarolo, F., ... & Tempia, F. (2019). Mice harbouring a SCA28 patient mutation in AFG3L2 develop late-onset ataxia associated with enhanced mitochondrial proteotoxicity. Neurobiology of disease, 124, 14-28.

Bettegazzi, B., Pelizzoni, I., Scarzella, F. S., Restelli, L. M., Zacchetti, D., Maltecca, F., ... & Codazzi, F. (2019). Upregulation of peroxiredoxin 3 protects afg3l2-KO cortical neurons in vitro from oxidative stress: a paradigm for neuronal cell survival under neurodegenerative conditions. Oxidative medicine and cellular longevity, 2019.

Cesnekova, J., Rodinova, M., Hansikova, H., Zeman, J., & Stiburek, L. (2018). Loss of mitochondrial AAA proteases AFG3L2 and YME1L impairs mitochondrial structure and respiratory chain biogenesis. International journal of molecular sciences, 19(12), 3930.

Ding, B., Martin, D. W., Rampello, A. J., & Glynn, S. E. (2018). Dissecting substrate specificities of the mitochondrial AFG3L2 protease. Biochemistry, 57(28), 4225-4235.

Seegobin, M. (2017). Examining Parkinson’s Disease Linked DJ-1 and its Interaction with Autophagy Related ATG5 and ATG12 & Understanding PINK1’s Functional Interaction with Mitochondrial m-AAA Protease AFG3L2 (Doctoral dissertation, Université d'Ottawa/University of Ottawa).

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

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