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品种反应性 主要应用 宿主 格式 抗体类型 H IHC, WB, ICC Rb Affinity Purified Polyclonal Antibody 描述 产品目录编号 06-1037 描述 Anti-TMEM126A Antibody Alternate Names - transmembrane protein 126A
背景信息 Transmembrane protein 126A (TMEM126A) is thought to be localized in the mitochondria however, the function is unknown. TMEM126A has been associated with mitochondrial dysfunction and nonsyndromic autosomal-recessive optic neuropathies. 产品信息 格式 Affinity Purified 控制 - Human liver tissue lysate.
演示 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide 应用 应用 Use Anti-TMEM126A Antibody (Rabbit Polyclonal Antibody) validated in WB, ICC, IHC to detect TMEM126A also known as transmembrane protein 126A. 主要应用 - Immunohistochemistry
- Western Blotting
- Immunocytochemistry
应用说明 Immunocytochemistry Analysis: A representative lot of this antibody was used by an independent laboratory in IC. (Korfali, N., et al. (2010). Mol Cell Proteomics. 9:2571-2585.)
Immunohistochemistry Analysis: A representative lot of this antibody was used by an independent laboratory in IH. (Korfali, N., et al. (2010). Mol Cell Proteomics. 9:2571-2585.)生物信息 免疫原品种 KLH-conjugated linear peptide corresponding to human TMEM126A. 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Rabbit 品种反应性 Human Species Reactivity Note Demonstrated to react with human. 抗体类型 Polyclonal Antibody Entrez基因编号 - NP_115649
基因符号 - TMEM126A
纯化方法 Affinity purified UniProt编号 - Q9H061
UniProt汇总 SUBCELLULAR LOCATION: Membrane; Multi-pass membrane protein (potential). Mitochondrion.
TISSUE SPECIFICITY: Strongly expressed in brain, cerebellum, skeletal muscle, testis. High expression also found in fetal brain, fetal retinal pigmentary epithelium, and fetal retina.
INVOLVEMENT IN DISEASE: Defects in TMEM126A are the cause of optic atrophy type 7 (OPA7) [MIM:612989]. A hereditary condition that features progressive visual loss in association with optic atrophy. Atrophy of the optic disk indicates a deficiency in the number of nerve fibers which arise in the retina and converge to form the optic disk, optic nerve, optic chiasm and optic tracts. OPA7 is an autosomal recessive juvenile-onset optic atrophy characterized by severe bilateral deficiency in visual acuity, optic disk pallor, and central scotoma.
SEQUENCE SIMILARITIES: Belongs to the TMEM126 family.产品使用声明 质量保证 Evaluated by Western Blot in human liver tissue lysate.
Western Blot Analysis: 0.125 µg/mL of this antibody detected TMEM126A on 10 µg of human liver tissue lysate.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 100 µg
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