Key features and details
- Rabbit polyclonal to MPV17
- Suitable for: ICC/IF, WB
- Reacts with: Mouse, Human
- Isotype: IgG
Product nameAnti-MPV17 antibody
DescriptionRabbit polyclonal to MPV17
Tested applicationsSuitable for: ICC/IF, WBmore details
Species reactivityReacts with: Mouse, Human
Predicted to work with: Rat, Cow
Recombinant full length protein corresponding to Human MPV17 aa 1-176.
MALWRAYQRALAAHPWKVQVLTAGSLMGLGDIISQQLVERRGLQEHQRGR TLTMVSLGCGFVGPVVGGWYKVLDRFIPGTTKVDALKKMLLDQGGFAPCF LGCFLPLVGALNGLSAQDNWAKLQRDYPDALITNYYLWPAVQLANFYLVP LHYRLAVVQCVAVIWNSYLSWKAHRL
Database link: P39210
- WB: Mouse heart tissue lysate. ICC/IF: HeLa cells.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.40
Constituents: 50% Glycerol (glycerin, glycerine), PBS, 0.03% Proclin 300
Concentration information loading...
PurityProtein G purified
Purification notesPurity >95%
Our Abpromise guarantee covers the use of ab236746 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||1/50 - 1/200.|
|WB||1/1000 - 1/5000. Predicted molecular weight: 20 kDa.|
FunctionInvolved in mitochondria homeostasis. May be involved in the metabolism of reactive oxygen species and control of oxidative phosphorylation and mitochondrial DNA (mtDNA) maintenance.
Tissue specificityUbiquitous. Expressed in pancreas, kidney, muscle, liver, lung, placenta, brain and heart.
Involvement in diseaseDefects in MPV17 are a cause of hepatocerebral mitochondrial DNA depletion syndrome (MDS) [MIM:251880]. MDS is a clinically heterogeneous group of disorders characterized by a reduction in mitochondrial DNA (mtDNA) copy number. Primary mtDNA depletion is inherited as an autosomal recessive trait and may affect single organs, typically muscle or liver, or multiple tissues. Individuals with the hepatocerebral form of mitochondrial DNA depletion syndrome have early progressive liver failure and neurologic abnormalities, hypoglycemia, and increased lactate in body fluids.
Defects in MPV17 are the cause of Navajo neurohepatopathy (NN) [MIM:256810]. NN is an autosomal recessive disease that is prevalent among Navajo children in the southwestern United States. The major clinical features are hepatopathy, peripheral neuropathy, corneal anesthesia and scarring, acral mutilation, cerebral leukoencephalopathy, failure to thrive, and recurrent metabolic acidosis with intercurrent infections. Infantile, childhood, and classic forms of NN have been described. Mitochondrial DNA depletion was detected in the livers of patients, suggesting a primary defect in mtDNA maintenance.
Sequence similaritiesBelongs to the peroxisomal membrane protein PXMP2/4 family.
Cellular localizationMitochondrion inner membrane.
- Information by UniProt
- Glomerulosclerosis antibody
- Mpv17 antibody
- Mpv17 human homolog of glomerulosclerosis and nephrotic syndrome antibody
Anti-MPV17 antibody (ab236746) at 1/1000 dilution + Mouse heart tissue lysate
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 20 kDa
HeLa (Human epithelial cell line from cervix adenocarcinoma) cells stained for MPV17 (green) using ab236746 at 1/100 dilution in ICC/IF. Secondary antibody is an Alexa-Fluor® 488-conjugated Goat Anti-Rabbit IgG (H+L).
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
ab236746 has not yet been referenced specifically in any publications.