TIMM8A
Reaktivität: Human
IF (cc), IF (p)
Wirt: Kaninchen
Polyclonal
AbBy Fluor® 488
Applikationshinweise
IHC 1:40-1:200, ELISA 1:5000-1:10000
Beschränkungen
Nur für Forschungszwecke einsetzbar
Format
Liquid
Konzentration
0.84 mg/mL
Buffer
PBS with 0.05 % Sodium azide and 40 % Glycerol, pH 7.4
Konservierungsmittel
Sodium azide
Vorsichtsmaßnahmen
This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Lagerung
-20 °C
Informationen zur Lagerung
Store at -20°C. Avoid freeze / thaw cycles.
Target
TIMM8A/DDP (TIMM8A)
(Translocase of Inner Mitochondrial Membrane 8A (TIMM8A))
Andere Bezeichnung
TIMM8A
Hintergrund
This translocase is involved in the import and insertion of hydrophobic membrane proteins from the cytoplasm into the mitochondrial inner membrane. The gene is mutated in Mohr-Tranebjaerg syndrome/Deafness Dystonia Syndrome (MTS/DDS) and it is postulated that MTS/DDS is a mitochondrial disease caused by a defective mitochondrial protein import system. Defects in this gene also cause Jensen syndrome, an X-linked disease with opticoacoustic nerve atrophy and muscle weakness. This protein, along with TIMM13, forms a 70 kDa heterohexamer. Alternative splicing results in multiple transcript variants encoding distinct isoforms.