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Product Name
MPV17 antibody
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Description
MPV17 Rabbit Polyclonal antibody. Positive IHC detected in human pancreas cancer tissue. Positive IF detected in HeLa cells. Positive WB detected in mouse kidney tissue, human brain tissue, human heart tissue, human liver tissue, human placenta tissue. Observed molecular weight by Western-blot: 20kd
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Tested applications
ELISA, WB, IHC, IF
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Species reactivity
Human, Mouse; other species not tested.
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Alternative names
MPV17 antibody; Protein Mpv17 antibody; SYM1 antibody
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Isotype
Rabbit IgG
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Preparation
This antibody was obtained by immunization of MPV17 recombinant protein (Accession Number: XM_005264326). 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: 1:200-1:2000
IHC: 1:20-1:200
IF: 1:10-1:100
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Validations
mouse kidney tissue were subjected to SDS PAGE followed by western blot with Catalog No:112757(MPV17 antibody) at dilution of 1:500
Immunohistochemical of paraffin-embedded human pancreas cancer using Catalog No:112757(MPV17 antibody) at dilution of 1:50 (under 10x lens)
Immunohistochemical of paraffin-embedded human pancreas cancer using Catalog No:112757(MPV17 antibody) at dilution of 1:50 (under 40x lens)
Immunofluorescent analysis of HeLa cells using Catalog No:112757(MPV17 Antibody) at dilution of 1:25 and Alexa Fluor 594-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
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Background
MPV17, also termed as SYM1, encodes an inner mitochondrial membrane protein. It’s potential candidate that may account for the mitochondrial (mt) DNA depletion syndromes (MDDS) characterized by a severe, tissue-specific decrease of mtDNA copy number and ultimate organ failure. It’s a human ortholog of the mouse kidney disease gene Mpv17. Absence or malfunction of MPV17 causes oxidative phosphorylation (OXPHOS) failure and mtDNA depletion, not only in affected individuals but also in Mpv17-/- mice. MPV17 contribute to ROS homeostasis, but the downstream signaling is unknown yet. Catalog# 10310-1-AP is a rabbit polyclonal antibody raised against the full-length of human MPV17.
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References
- Casalena G, Krick S, Daehn I. Mpv17 in mitochondria protects podocytes against mitochondrial dysfunction and apoptosis in vivo and in vitro. American journal of physiology. Renal physiology. 306(11):F1372-80. 2014.
- Spinazzola A, Viscomi C, Fernandez-Vizarra E. MPV17 encodes an inner mitochondrial membrane protein and is mutated in infantile hepatic mitochondrial DNA depletion. Nature genetics. 38(5):570-5. 2006.
- Bottani E, Giordano C, Civiletto G. AAV-mediated liver-specific MPV17 expression restores mtDNA levels and prevents diet-induced liver failure. Molecular therapy : the journal of the American Society of Gene Therapy. 22(1):10-7. 2014.
- Navarro-Sastre A, Martín-Hernández E, Campos Y. Lethal hepatopathy and leukodystrophy caused by a novel mutation in MPV17 gene: description of an alternative MPV17 spliced form. Molecular genetics and metabolism. 94(2):234-9. 2008.
- Dalla Rosa I, Durigon R, Pearce SF. MPV17L2 is required for ribosome assembly in mitochondria. Nucleic acids research. 42(13):8500-15. 2014.
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