Rabbit anti-Human, Mouse POLG Polyclonal Antibody | anti-POLG antibody

POLG, CT (POLG, MDP1, POLG1, POLGA, DNA polymerase subunit gamma-1, Mitochondrial DNA polymerase catalytic subunit, PolG-alpha)

Cat. Num. AAA648067

• Gene Names: POLG; PEO; MDP1; SCAE; MIRAS; POLG1; POLGA; SANDO; MTDPS4A; MTDPS4B
• Reactivity: Human, Mouse
• Applications: ELISA, Western Blot, Immunohistochemistry, Flow Cytometry, Functional Assay, Immunofluorescence
• Purity: Affinity Purified; Purified by Protein A affinity chromatography.
• Synonyms: POLG; Polyclonal Antibody; CT (POLG; MDP1; POLG1; POLGA; DNA polymerase subunit gamma-1; Mitochondrial DNA polymerase catalytic subunit; PolG-alpha); Anti -POLG; anti-POLG antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Human, mouse (predicted)
• Purity/Purification: Affinity Purified; Purified by Protein A affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

• Applicable Applications for anti-POLG antibody: ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC), Flow Cytometry (FC/FACS), Immunofluorescence (IF)
• Application Notes: Suitable for use in Western Blot, Immunohistochemistry, Immunofluorescence, Flow Cytometry, ELISA; Dilution: ELISA: 1:1,000; Western Blot: 1:100-500; Flow Cytometry: 1:10-50; Immunofluorescence: 1:10-50; Immunohistochemistry: 1:10-50
• Immunogen: POLG antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 1119-1148 amino acids from the C-terminal region of human POLG.
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western blot analysis in mouse cerebellum tissue lysates (35ug/lane).This demonstrates the MBS648067 antibody detected the POLG protein (arrow).)

Flow Cytometry (FC/FACS)

(MBS648067 flow cytometric analysis of CEM cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Immunofluorescence (IF)

(Confocal immunofluorescent analysis of MBS648067 with Hela cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green).DAPI was used to stain the cell nuclear (blue).)

Immunohistochemistry (IHC)

(MBS648067 immunohistochemistry analysis in formalin fixed and paraffin embedded human skeletal muscle followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of MBS648067 for immunohistochemistry. Clinical relevance has not been evaluated.)

Related Product Information for anti-POLG antibody

Mitochondrial DNA polymerase is heterotrimeric, consisting of a homodimer of accessory subunits plus a catalytic subunit. The protein encoded by this gene is the catalytic subunit of mitochondrial DNA polymerase. The encoded protein contains a polyglutamine tract near its N-terminus that may be polymorphic. Defects in this gene are a cause of progressive external ophthalmoplegia with mitochondrial DNA deletions 1 (PEOA1), sensory ataxic neuropathy dysarthria and ophthalmoparesis (SANDO), Alpers-Huttenlocher syndrome (AHS), and mitochondrial neurogastrointestinal encephalopathy syndrome (MNGIE). Two transcript variants encoding the same protein have been found for this gene.

Product Categories/Family for anti-POLG antibody

Antibodies; Abs to Mitochondrial Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 29791648
• NCBI GeneID: 5428
• UniProt Accession #: P54098; Q8NFM2; Q92515
• Molecular Weight: 139,562 Da
• NCBI Official Full Name: POLG protein
• NCBI Official Synonym Full Names: polymerase (DNA directed), gamma
• NCBI Official Symbol: POLG
• NCBI Official Synonym Symbols: PEO; MDP1; SCAE; MIRAS; POLG1; POLGA; SANDO; MTDPS4A; MTDPS4B
• NCBI Protein Information: DNA polymerase subunit gamma-1; PolG-alpha; mitochondrial DNA polymerase catalytic subunit
• UniProt Protein Name: DNA polymerase subunit gamma-1
• Protein Family: DNA polymerase
• UniProt Gene Name: POLG
• UniProt Synonym Gene Names: MDP1; POLG1; POLGA
• UniProt Entry Name: DPOG1_HUMAN

NCBI Description

Mitochondrial DNA polymerase is heterotrimeric, consisting of a homodimer of accessory subunits plus a catalytic subunit. The protein encoded by this gene is the catalytic subunit of mitochondrial DNA polymerase. The encoded protein contains a polyglutamine tract near its N-terminus that may be polymorphic. Defects in this gene are a cause of progressive external ophthalmoplegia with mitochondrial DNA deletions 1 (PEOA1), sensory ataxic neuropathy dysarthria and ophthalmoparesis (SANDO), Alpers-Huttenlocher syndrome (AHS), and mitochondrial neurogastrointestinal encephalopathy syndrome (MNGIE). Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008]

Uniprot Description

Function: Involved in the replication of mitochondrial DNA. Associates with mitochondrial DNA.

Catalytic activity: Deoxynucleoside triphosphate + DNA(n) = diphosphate + DNA(n+1).

Cofactor: Magnesium.

Subunit structure: Heterotrimer composed of a catalytic subunit and a homodimer of accessory subunits.

Subcellular location: Mitochondrion. Mitochondrion matrix › mitochondrion nucleoid Ref.6.

Polymorphism: The poly-Gln region seems to be polymorphic.

Involvement in disease: Progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal dominant, 1 (PEOA1) [MIM:157640]: A disorder 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.8 Ref.9 Ref.19 Ref.20 Ref.34 Ref.35Progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal recessive (PEOB) [MIM:258450]: A severe form of progressive external ophthalmoplegia. It is clinically more heterogeneous than the autosomal dominant forms.Note: The disease is caused by mutations affecting the gene represented in this entry.Sensory ataxic neuropathy dysarthria and ophthalmoparesis (SANDO) [MIM:607459]: A systemic disorder resulting from mitochondrial dysfunction associated with mitochondrial depletion in skeletal muscle and peripheral nerve tissue. The clinical triad of symptoms consists of sensory ataxic neuropathy, dysarthria, and ophthalmoparesis. However, the phenotype varies widely, even within the same family, and can also include myopathy, seizures, and hearing loss. An atypical form of the disease is characterized by headaches and/or seizures manifesting in childhood or adolescence, followed by development of cerebellar and sensory ataxia, dysarthria, progressive external ophthalmoplegia, and myoclonus in early adulthood.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.16 Ref.21 Ref.22 Ref.23 Ref.26 Ref.27 Ref.30 Ref.31 Ref.33Mitochondrial DNA depletion syndrome 4A (MTDPS4A) [MIM:203700]: An autosomal recessive hepatocerebral syndrome due to mitochondrial dysfunction. The typical course of the disease 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 are neuronal loss, spongiform degeneration, and astrocytosis of the visual cortex. Liver biopsy results show steatosis, often progressing to cirrhosis.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.17 Ref.24 Ref.25 Ref.30 Ref.31 Ref.36Mitochondrial DNA depletion syndrome 4B (MTDPS4B) [MIM:613662]: An autosomal recessive progressive multisystem disorder due to mitochondrial dysfunction. It is clinically characterized by chronic gastrointestinal dysmotility and pseudo-obstruction, cachexia, progressive external ophthalmoplegia, axonal sensory ataxic neuropathy, and muscle weakness.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.11 Ref.37Leigh syndrome (LS) [MIM:256000]: An early-onset progressive neurodegenerative disorder characterized by the presence of focal, bilateral lesions in one or more areas of the central nervous system including the brainstem, thalamus, basal ganglia, cerebellum and spinal cord. Clinical features depend on which areas of the central nervous system are involved and include subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, and dysphagia.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.36

Sequence similarities: Belongs to the DNA polymerase type-A family.