Overview

  • Product name
    Anti-MPV17 antibody
  • Description
    Goat polyclonal to MPV17
  • Tested applications
    Suitable for: IHC-P, WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Rat, Cow, Dog
  • Immunogen

    Synthetic peptide:

    C-QRALAAHPWKVQ

    , corresponding to internal sequence amino acids 8-19 of Human MPV17 (NP_002428.1).

  • Positive control
    • Human Brain (frontal cortex) lysates.

Properties

Applications

Our Abpromise guarantee covers the use of ab109583 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
IHC-P Use a concentration of 5 - 10 µg/ml. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
WB Use a concentration of 1 - 3 µg/ml. Detects a band of approximately 19 kDa (predicted molecular weight: 20 kDa).

Target

  • Function
    Involved in mitochondria homeostasis. May be involved in the metabolism of reactive oxygen species and control of oxidative phosphorylation and mitochondrial DNA (mtDNA) maintenance.
  • Tissue specificity
    Ubiquitous. Expressed in pancreas, kidney, muscle, liver, lung, placenta, brain and heart.
  • Involvement in disease
    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.
  • Sequence similarities
    Belongs to the peroxisomal membrane protein PXMP2/4 family.
  • Cellular localization
    Mitochondrion inner membrane.
  • Information by UniProt
  • Database links
  • Alternative names
    • Glomerulosclerosis antibody
    • Mpv17 antibody
    • Mpv17 human homolog of glomerulosclerosis and nephrotic syndrome antibody
    • MpV17 mitochondrial inner membrane protein antibody
    • MPV17_HUMAN antibody
    • MTDPS6 antibody
    • Protein Mpv17 antibody
    • SYM1 antibody
    see all

Images

  • 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.

  • 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.

References

ab109583 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

Application
Western blot
Sample
Mouse Tissue lysate - whole (Brain homogenate, and mitochondria isolated)
Gel Running Conditions
Reduced Denaturing (4-12%)
Loading amount
20 µg
Specification
Brain homogenate, and mitochondria isolated
Blocking step
SEA Block Blocking Buffer as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 100% · Temperature: 22°C
Username

Svitlana Yablonska

Verified customer

Submitted Jun 21 2017

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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