Overview

  • Product name

  • Description

    Rabbit polyclonal to MPV17
  • Host species

    Rabbit
  • Tested applications

    Suitable for: ICC/IF, WBmore details
  • Species reactivity

    Reacts with: Mouse, Human
    Predicted to work with: Rat, Cow
  • Immunogen

    Recombinant full length protein corresponding to Human MPV17 aa 1-176.
    Sequence:

    MALWRAYQRALAAHPWKVQVLTAGSLMGLGDIISQQLVERRGLQEHQRGR TLTMVSLGCGFVGPVVGGWYKVLDRFIPGTTKVDALKKMLLDQGGFAPCF LGCFLPLVGALNGLSAQDNWAKLQRDYPDALITNYYLWPAVQLANFYLVP LHYRLAVVQCVAVIWNSYLSWKAHRL


    Database link: P39210

  • Positive control

    • WB: Mouse heart tissue lysate. ICC/IF: HeLa cells.

Properties

Applications

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.

Application Abreviews Notes
ICC/IF 1/50 - 1/200.
WB 1/1000 - 1/5000. 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

  • Anti-MPV17 antibody (ab236746) at 1/1000 dilution + Mouse heart tissue lysate

    Secondary
    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).

References

ab236746 has not yet been referenced specifically in any publications.

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