Product DescriptionMouse anti-Deafness dystonia protein 1 Monoclonal Antibody (Unconjugated), suitable for WB, IHC-Frozen, ELISA.
Application(s)ELISA, IHC-Frozen, WB
Application DetailsThis antibody is recommended for WB, IHC and sandwich ELISA. For IHC, a concentration of 3 µg/mL is recommended. Biosensis recommends optimal dilutions/concentrations should be determined by the end user.
TargetDeafness dystonia protein 1
SpecificitySpecificity has been confirmed by WB and direct ELISA against the antigen. Human. Other species have not been tested.
Target Host SpeciesHuman
Immunogen DescriptionPartial recombinant protein of human Deafness dystonia protein 1 (aa 9 to 98) with a GST tag.
Purity DescriptionProtein G purified immunoglobulin
FormatLyophilized from PBS pH 7.2
Reconstitution InstructionsSpin vial briefly before opening. Reconstitute in 100 µL sterile-filtered, ultrapure water. Centrifuge to remove any insoluble material.
Storage InstructionsAfter reconstitution keep aliquots at -20°C for higher stability or at 2-8°C with an appropriate antibacterial agent. Glycerol (1:1) may be added for additional stability. Avoid repetitive freeze/thaw cycles.
Batch NumberPlease see item label.
Expiration Date12 months after date of receipt (unopened vial).
TIMM8A is a mitochondrial intermembrane chaperone that is involved in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. It also acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space. TIMM8A is probably necessary for normal neurologic development. TIMM8A is a heterohexamer composed of three TIMM8A and three TIMM13 subunits, named soluble 70KDa complex. It associates with the TIMM22 complex. It is located at the mitochondrion inner membrane and is highly expressed in fetal and adult brain, followed by fetal lung, liver and kidney. Defects in TIMM8A cause Mohr-Tranebjaerg syndrome (MTS) also known as dystonia-deafness syndrome (DDS) or X-linked progressive deafnes type 1 (DFN-1). This is a recessive neurodegenerative syndrome characterised by progressive deafness, dystonia, spasticity, dysphagia, mental deterioration, paranoia and cortical blindness. Defects in TIMM8A are also the cause of Jensen syndrom, also known as opticoacoustic nerve atrophy with dementia. This is an X-linked disease characterised by deafness, blindess and muscle weakness.
Immunohistochemical detection of Deafness dystonia protein 1 in paraffin-embedded, formalin fixed human liver tissue using mouse monoclonal antibody to Deafness dystonia protein 1, catalogue number M-831-100.
Western blot detection of Deafness dystonia protein 1 expression in HeLa cell lysate using mouse monoclonal antibody to human Deafness dystonia protein 1, catalogue number M-831-100.