The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 5 - 10 µg/ml. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
Use a concentration of 1 - 3 µg/ml. Detects a band of approximately 19 kDa (predicted molecular weight: 20 kDa).
功能Involved in mitochondria homeostasis. May be involved in the metabolism of reactive oxygen species and control of oxidative phosphorylation and mitochondrial DNA (mtDNA) maintenance.
组织特异性Ubiquitous. Expressed in pancreas, kidney, muscle, liver, lung, placenta, brain and heart.
疾病相关Defects 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.
序列相似性Belongs to the peroxisomal membrane protein PXMP2/4 family.
ab109583 (5µg/ml) shows staining of MPV17 in paraffin embedded Human Liver following steamed antigen retrieval with citrate buffer pH 6 and AP-staining. This shows heavy textured cystoplasm staining in hepatocytes, consistent with mitochondia.
Western blot - MPV17 antibody (ab109583)
Anti-MPV17 antibody (ab109583) at 2 µg/ml + Human Frontal Cortex lysate (in RIPA buffer) at 35 µg
Predicted band size : 20 kDa Additional bands at : 26 kDa. We are unsure as to the identity of these extra bands.An additional band of unknown identity was consistently observed at 26kDa. This band was successfully blocked by incubation with the immunizing peptide.
Anti-MPV17 antibody (ab109583)参考文献
has not yet been referenced specifically in any publications.