Recombinant
RabMAb

Recombinant Anti-POLG antibody [EPR7296] - BSA and Azide free (ab232583)

Overview

  • Product name

    Anti-POLG antibody [EPR7296] - BSA and Azide free
    See all POLG primary antibodies
  • Description

    Rabbit monoclonal [EPR7296] to POLG - BSA and Azide free
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide within Human POLG aa 1150-1200. The exact sequence is proprietary.

  • Positive control

    • WB: HepG2 and HEK-293T cell lysate.
  • General notes

    Ab232583 is the carrier-free version of ab128899. This format is designed for use in antibody labeling, including fluorochromes, metal isotopes, oligonucleotides, enzymes.

     

    Our carrier-free formats are supplied in a buffer free of BSA, sodium azide and glycerol for higher conjugation efficiency.

    Use our conjugation kits  for antibody conjugates that are ready-to-use in as little as 20 minutes with <1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.

    ab232583 is compatible with the Maxpar® Antibody Labeling Kit from Fluidigm.

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab232583 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
WB Use at an assay dependent concentration. Predicted molecular weight: 140 kDa.

Target

  • Function

    Involved in the replication of mitochondrial DNA.
  • Involvement in disease

    Defects in POLG are the cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant type 1 (PEOA1) [MIM:157640]. Progressive external ophthalmoplegia is characterized by progressive weakness of ocular muscles and levator muscle of the upper eyelid. In a minority of cases, it is associated with skeletal myopathy, which predominantly involves axial or proximal muscles and which causes abnormal fatigability and even permanent muscle weakness. Ragged-red fibers and atrophy are found on muscle biopsy. A large proportion of chronic ophthalmoplegias are associated with other symptoms, leading to a multisystemic pattern of this disease. Additional symptoms are variable, and may include cataracts, hearing loss, sensory axonal neuropathy, ataxia, depression, hypogonadism, and parkinsonism.
    Defects in POLG are a cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal recessive (PEOB) [MIM:258450]. PEOB is a severe form of progressive external ophthalmoplegia. It is clinically more heterogeneous than the autosomal dominant forms. Can be more severe.
    Defects in POLG are a cause of sensory ataxic neuropathy dysarthria and ophthalmoparesis (SANDO) [MIM:607459]. SANDO is a clinically heterogeneous systemic disorder with variable features resulting from mitochondrial dysfunction. It shares phenotypic characteristics with autosomal recessive progressive external ophthalmoplegia and mitochondrial neurogastrointestinal encephalopathy syndrome. The clinical triad of symptoms consists of sensory ataxic, neuropathy, dysarthria, and ophthalmoparesis.
    Defects in POLG are a cause of Alpers-Huttenlocher syndrome (AHS) [MIM:203700]; also called Alpers diffuse degeneration of cerebral gray matter with hepatic cirrhosis. AHS is an autosomal recessive hepatocerebral syndrome. The typical course of AHS includes severe developmental delay, intractable seizures, liver failure, and death in childhood. Refractory seizures, cortical blindness, progressive liver dysfunction, and acute liver failure after exposure to valproic acid are considered diagnostic features. The neuropathological hallmarks of AHS are neuronal loss, spongiform degeneration, and astrocytosis of the visual cortex. Liver biopsy results show steatosis, often progressing to cirrhosis.
    Defects in POLG are a cause of mitochondrial neurogastrointestinal encephalopathy syndrome (MNGIE) [MIM:603041]; also known as myoneurogastrointestinal encephalomyopathy. MNGIE is an autosomal recessive disease associated with multiple deletions of skeletal muscle mitochondrial DNA (MtDNA). It is clinically characterized by onset between the second and fifth decades of life, ptosis, progressive external ophthalmoplegia, gastrointestinal dysmotility (often pseudoobstruction), diffuse leukoencephalopathy, thin body habitus, peripheral neuropathy, and myopathy.
    Defects in POLG are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.
  • Sequence similarities

    Belongs to the DNA polymerase type-A family.
  • Cellular localization

    Mitochondrion.
  • Information by UniProt
  • Database links

  • Alternative names

    • DNA directed DNA polymerase gamma antibody
    • DNA polymerase subunit gamma 1 antibody
    • DNA polymerase subunit gamma-1 antibody
    • DPOG1_HUMAN antibody
    • MDP 1 antibody
    • MDP1 antibody
    • Mitochondrial DNA polymerase catalytic subunit antibody
    • Mitochondrial DNA polymerase gamma antibody
    • PEO antibody
    • POLG 1 antibody
    • POLG A antibody
    • PolG alpha antibody
    • POLG antibody
    • PolG-alpha antibody
    • POLG1 antibody
    • POLGA antibody
    • Polymerase (DNA directed) gamma antibody
    • SANDO antibody
    • SCAE antibody
    see all

Images

  • Equilibrium disassociation constant (KD)
    Learn more about KD

    Click here to learn more about KD

    This data was developed using the same antibody clone in a different buffer formulation containing PBS, BSA, glycerol, and sodium azide (ab128899).

  • All lanes : Anti-POLG antibody [EPR7296] (ab128899) at 1/5000 dilution (purified)

    Lane 1 : HEK-293T (human embryonic kidney) whole cell lysate
    Lane 2 : HepG2 (Human liver hepatocellular carcinoma cell line) whole cell lysate

    Lysates/proteins at 20 µg per lane.

    Secondary
    All lanes : Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/20000 dilution

    Predicted band size: 140 kDa



    Blocking and diluting buffer: 5% NFDM/TBST.

    This data was developed using the same antibody clone in a different buffer formulation containing PBS, BSA, glycerol, and sodium azide (ab128899).

References

ab232583 has not yet been referenced specifically in any publications.

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