| 克隆類型 ： |
Rabbit Polyclonal IgG |
|
免 疫 原 ： |
Peptide derived from Hu ATP carrier protein 1/Adenine Nucleotide Translocase 1. |
|
純化方式 ： |
Immunogen affinity purified |
|
緩 沖 液 ： |
PBS, pH 7.4(1%BSA and 0.1% Sodium azide) |
|
產(chǎn)品描述 ： |
Defects in SLC25A4 are a cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant type 2 (PEOA2) [MIM:609283]. 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. |
| 適用物種 ： |
Hu, Mo, Rat, Dog, Pig, Cow, Sheep, |
| 應(yīng)用說明 ： |
WB: 1:200-1:1000 E: 1:500-1:1000 IHC: 1:200-1:1000 IF: 1:100-1:500 |
| 別 名 ： |
heart/skeletal muscle isoform T1; Adenine nucleotide translocator 1 (skeletal muscle); Adenine nucleotide translocator 1; ADP; ADP ATP carrier protein 1; ADP ATP carrier protein heart/skeletal muscle isoform T1; ADP/ATP translocase 1; ADT1_Hu; ANT; ANT1; ATP carrier protein 1; ATP carrier protein; MSA02; PEO2; PEO3; SLC25A4; Solute carrier family 25 member 4; T1 antibody. |
| 保存條件 ： |
Store at -20°C for one year. Avoid repeated freeze-thaw cycles. At 4°C for 2-3 weeks after reconstituted. |
| 標(biāo) 記 物 ： |
PLLABS公司可供應(yīng)生物素、辣根過氧化物酶、熒光素標(biāo)記抗體 |
|
產(chǎn)品說明 ： |
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