货号：06-1037

品牌：Merck Millipore

规格：

目录价：￥4259.00

市场价格：￥3620.15

会员价格：￥3407.20

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-TMEM126A Antibody | 06-1037 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Molecular Weight: ~23 kDa observed View All» Immunogen: KLH-conjugated linear peptide corresponding to human TMEM126A. View All» Background Information: 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. View All» Species Reactivity: Human View All» Species Reactivity Note: Demonstrated to react with human. View All» Application Notes: 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.) View All» Control: Human liver tissue lysate. View All» Quality Assurance: 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. View All» Purification Method: Affinity purified View All» Presentation: 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide View All» Storage Conditions: Stable for 1 year at 2-8°C from date of receipt. View All» UniProt Number: Q9H061 View All» Entrez Gene Number: NP_115649 View All» Gene Symbol: TMEM126A View All» Alternate Names: transmembrane protein 126A View All» Usage Statement: 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. View All» Key Applications:

• Western Blotting

• Immunocytochemistry

• Immunohistochemistry
  View All» UniProt Summary: 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. View All» Product Name: Anti-TMEM126A View All» Concentration: 1 mg/mL View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Affinity Purified View All» Host: Rabbit View All»
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