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Product Name
AFG3L2 antibody
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Description
AFG3L2 Rabbit Polyclonal antibody. Positive IP detected in mouse kidney tissue. Positive WB detected in HeLa cells, HEK-293 cells, human brain tissue, mouse brain tissue, mouse heart tissue, mouse kidney tissue, mouse liver tissue, mouse skeletal muscle tissue. Positive IHC detected in human kidney tissue. Positive IF detected in HepG2 cells. Observed molecular weight by Western-blot: 80-90 kDa
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Tested applications
ELISA, WB, IHC, IF, IP
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Species reactivity
Human,Mouse,Rat; other species not tested.
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Alternative names
AFG3 like protein 2 antibody; AFG3L2 antibody; FLJ25993 antibody; Paraplegin like protein antibody
- Immunogen
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Isotype
Rabbit IgG
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Preparation
This antibody was obtained by immunization of AFG3L2 recombinant protein (Accession Number: NM_006796). Purification method: Antigen affinity purified.
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Clonality
Polyclonal
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Formulation
PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
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Storage instructions
Store at -20℃. DO NOT ALIQUOT
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Applications
Recommended Dilution:
WB: 1:500-1:5000
IP: 1:500-1:5000
IHC: 1:20-1:200
IF: 1:10-1:100
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Validations
HeLa cells were subjected to SDS PAGE followed by western blot with Catalog No:107908(AFG3L2 antibody) at dilution of 1:800
Immunohistochemical of paraffin-embedded human kidney using Catalog No:107908(AFG3L2 antibody) at dilution of 1:50 (under 10x lens)
Immunohistochemical of paraffin-embedded human kidney using Catalog No:107908(AFG3L2 antibody) at dilution of 1:50 (under 40x lens)
Immunofluorescent analysis of HepG2 cells, using AFG3L2 antibody Catalog No:107908 at 1:25 dilution and Rhodamine-labeled goat anti-rabbit IgG (red).
IP Result of anti-AFG3L2 (IP:Catalog No:107908, 4ug; Detection:Catalog No:107908 1:1000) with mouse kidney tissue lysate 4000ug.
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Background
AFG3L2 is the catalytic subunit of the m-AAA protease, an ATP-dependent proteolytic complex of the mitochondrial inner membrane that degrades misfolded proteins and regulates ribosome assembly(PMID:17101804). Human AFG3L2 is an 80-kDa protein encoded by a 17-exon gene and highly and selectively expressed in human cerebellar Purkinje cells(PMID:20208537) and it can exsit as a truncated 65 kDa protein(PMID:18337413). Defects in AFG3L2 are the cause of spinocerebellar ataxia type 28 (SCA28) and spastic ataxia autosomal recessive type 5 (SPAX5).
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References
- Pfeffer G, Gorman GS, Griffin H. Mutations in the SPG7 gene cause chronic progressive external ophthalmoplegia through disordered mitochondrial DNA maintenance. Brain : a journal of neurology. 137(Pt 5):1323-36. 2014.
- Richter U, Lahtinen T, Marttinen P, Suomi F, Battersby BJ. Quality control of mitochondrial protein synthesis is required for membrane integrity and cell fitness. The Journal of cell biology. 211(2):373-89. 2015.
- Gorman GS, Blakely EL, Hornig-Do HT. Novel MTND1 mutations cause isolated exercise intolerance, complex I deficiency and increased assembly factor expression. Clinical science (London, England : 1979). 128(12):895-904. 2015.
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