A synthetic peptide from aa region 10-60 of human TMEM126A conjugated to blue carrier protein was used as the antigen.
Transmembrane protein 126A
SUBCELLULAR LOCATION: Mitochondrion inner membrane; Multi-pass membrane protein
TISSUE SPECIFICITY: Strongly expressed in brain, cerebellum, skeletal muscle, testis. High expression also found in fetal brain, fetal retinal pigmentary epithelium, and fetal retina. Highly expressed in retinal ganglion cells.
Involvement in disease: Optic atrophy 7 (OPA7): 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.
Note: The disease is caused by mutations affecting the gene represented in this entry.
Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.
12 months after reconstitution
This item will be shipped to you at ambient temperature in a lyophilised form.
1. Hanein S, et al. Am. J. Hum. Genet. 84:493-498(2009)
For research use only
NZ white rabbit
Polyclonal, whole serum
IHC, WB (confirmed by recombinant protein). A dilution of 1: 300 to 1: 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.
Specific for TMEM126A.
Human. Other species not yet tested.
Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.