Recombinant
RabMAb

Recombinant Anti-POLG antibody [EPR7295] - BSA and Azide free (ab248192)

Overview

  • Product name

    Anti-POLG antibody [EPR7295] - BSA and Azide free
    See all POLG primary antibodies
  • Description

    Rabbit monoclonal [EPR7295] to POLG - BSA and Azide free
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WBmore details
    Unsuitable for: Flow Cyt,ICC/IF,IHC-P or IP
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Synthetic peptide within Human POLG aa 50-150. The exact sequence is proprietary.

  • General notes

    ab248192 is the carrier-free version of ab128862 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.

    Ab248192 is compatible with the Maxpar® Antibody Labeling Kit from Fluidigm.

    Maxpar® is a trademark of Fluidigm Canada Inc.

    Mouse, Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information. This product was previously labelled as DNA Polymerase gamma

    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

Associated products

Applications

Our Abpromise guarantee covers the use of ab248192 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. Detects a band of approximately 140 kDa (predicted molecular weight: 140 kDa).
  • Application notes
    Is unsuitable for Flow Cyt,ICC/IF,IHC-P or IP.
  • 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

    References

    ab248192 has not yet been referenced specifically in any publications.

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