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AFG3L2 Antibody (N-term)

Affinity Purified Rabbit Polyclonal Antibody (Pab)

     
  • WB - AFG3L2 Antibody (N-term) AP13219a
    AFG3L2 Antibody (N-term) (Cat. #AP13219a) western blot analysis in MDA-MB453,MCF-7,Jurkat cell line lysates (35ug/lane).This demonstrates the AFG3L2 antibody detected the AFG3L2 protein (arrow).
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  • IHC-P - AFG3L2 Antibody (N-term) AP13219a
    AFG3L2 Antibody (N-term) (Cat. #AP13219a)immunohistochemistry analysis in formalin fixed and paraffin embedded human cerebellum tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of AFG3L2 Antibody (N-term) for immunohistochemistry. Clinical relevance has not been evaluated.
    detail
  • SPECIFICATION
  • CITATIONS: 1
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  • BACKGROUND
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Product Information
Application
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • E=ELISA
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immunoelectronmicroscopy
  • EIA=Enzyme Immunoassay
IHC-P, WB, E
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
Additional Information
Gene ID 10939
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.
StorageMaintain 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.
PrecautionsAFG3L2 Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
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.
Research Areas
Citations ( 0 )

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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$ 365.00
$ 140.00
Cat# AP13219a
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