POLG2 Antibody (PACO41782)
- SKU:
- PACO41782
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | POLG2 Antibody (PACO41782) |
抗体コード: | PACO41782 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human DNA polymerase subunit gamma-2, mitochondrial protein (1-485AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
バックグラウンド: | Mitochondrial polymerase processivity subunit. Stimulates the polymerase and exonuclease activities, and increases the processivity of the enzyme. Binds to ss-DNA. |
シノニム: | DNA polymerase subunit gamma-2, mitochondrial (EC 2.7.7.7) (DNA polymerase γ accessory 55 kDa subunit) (p55) (Mitochondrial DNA polymerase accessory subunit) (MtPolB) (PolG-beta), POLG2, MTPOLB |
UniProt Protein Function: | POLG2: Mitochondrial polymerase processivity subunit. Stimulates the polymerase and exonuclease activities, and increases the processivity of the enzyme. Binds to ss-DNA. Defects in POLG2 are the cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant type 4 (PEOA4). 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. |
UniProt Protein Details: | Protein type:DNA replication; EC 2.7.7.7; Transferase; Mitochondrial; DNA repair, damage Chromosomal Location of Human Ortholog: 17q Cellular Component: mitochondrial matrix Molecular Function:glycine-tRNA ligase activity; protein binding Biological Process: DNA-dependent DNA replication; glycyl-tRNA aminoacylation; mitochondrion organization and biogenesis Disease: Progressive External Ophthalmoplegia With Mitochondrial Dna Deletions, Autosomal Dominant, 4 |
NCBI Summary: | This gene encodes the processivity subunit of the mitochondrial DNA polymerase gamma. The encoded protein forms a heterotrimer containing one catalytic subunit and two processivity subunits. This protein enhances DNA binding and promotes processive DNA synthesis. Mutations in this gene result in autosomal dominant progressive external ophthalmoplegia with mitochondrial DNA deletions.[provided by RefSeq, Sep 2009] |
UniProt Code: | Q9UHN1 |
NCBI GenInfo Identifier: | 17367139 |
NCBI Gene ID: | 11232 |
NCBI Accession: | Q9UHN1.1 |
UniProt Secondary Accession: | Q9UHN1,O00419, Q0IJ81, Q96GW2, Q9UK35, Q9UK94, |
UniProt Related Accession: | Q9UHN1 |
Molecular Weight: | 54,911 Da |
NCBI Full Name: | DNA polymerase subunit gamma-2, mitochondrial |
NCBI Synonym Full Names: | DNA polymerase gamma 2, accessory subunit |
NCBI Official Symbol: | POLG2 |
NCBI Official Synonym Symbols: | HP55; POLB; PEOA4; POLGB; MTPOLB; POLG-BETA |
NCBI Protein Information: | DNA polymerase subunit gamma-2, mitochondrial |
UniProt Protein Name: | DNA polymerase subunit gamma-2, mitochondrial |
UniProt Synonym Protein Names: | DNA polymerase gamma accessory 55 kDa subunit; p55; Mitochondrial DNA polymerase accessory subunit; MtPolB; PolG-beta |
Protein Family: | DNA polymerase |
UniProt Gene Name: | POLG2 |
UniProt Entry Name: | DPOG2_HUMAN |