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Product Name
TMEM70 antibody
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Description
TMEM70 Rabbit Polyclonal antibody. Positive IHC detected in human heart tissue. Positive WB detected in A2780 cells. Observed molecular weight by Western-blot: 18 kDa
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Tested applications
ELISA, WB, IHC
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Species reactivity
Human; other species not tested.
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Alternative names
FLJ20533 antibody; TMEM70 antibody; transmembrane protein 70 antibody
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Isotype
Rabbit IgG
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Preparation
This antibody was obtained by immunization of TMEM70 recombinant protein (Accession Number: NM_017866). 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: 1:1000-1:10000
IHC: 1:20-1:200
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Validations
A2780 cells were subjected to SDS PAGE followed by western blot with Catalog No:116195(TMEM70 antibody) at dilution of 1:800
Immunohistochemical of paraffin-embedded human heart using Catalog No:116195(TMEM70 antibody) at dilution of 1:100 (under 10x lens)
Immunohistochemical of paraffin-embedded human heart using Catalog No:116195(TMEM70 antibody) at dilution of 1:100 (under 40x lens)
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Background
TMEM70 belongs to the TMEM70 family. It is involved in biogenesis of mitochondrial ATP synthase. Defects in TMEM70 are a cause of mitochondrial encephalocardiomyopathy neonatal due to ATP synthase deficiency (MT-ATPSD). A publication(PMID:21147908) identifies that TMEM70 gene defect as a pan-ethnic disorder and further redefines it as the most common cause of nuclear-origin ATP synthase deficiency.
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References
- Torraco A, Verrigni D, Rizza T. TMEM70: a mutational hot spot in nuclear ATP synthase deficiency with a pivotal role in complex V biogenesis. Neurogenetics. 13(4):375-86. 2012.
- Kratochvílová H, Hejzlarová K, Vrbacký M. Mitochondrial membrane assembly of TMEM70 protein. Mitochondrion. 15:1-9. 2014.
- Diodato D, Invernizzi F, Lamantea E. Common and Novel TMEM70 Mutations in a Cohort of Italian Patients with Mitochondrial Encephalocardiomyopathy. JIMD reports. 15:71-8. 2015.
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