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

Name: Mouse Anti-AFG3L2 Recombinant Antibody (V2-12537)
SKU: CBMAB-1027-CN
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

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

Basic Information

Host Animal

Mouse

Clone

V2-12537

Application

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.

Application	Note
WB	1:500

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

Format

Liquid

Buffer

PBS, pH 7.3, 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

More Infomation

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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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)
Proteogenomic
Other Protocols

Isotype control

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

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