TIMM8A
Reactivité: Humain
IF (cc), IF (p)
Hôte: Lapin
Polyclonal
AbBy Fluor® 488
Indications d'application
IHC 1:40-1:200, ELISA 1:5000-1:10000
Restrictions
For Research Use only
Format
Liquid
Concentration
0.84 mg/mL
Buffer
PBS with 0.05 % Sodium azide and 40 % Glycerol, pH 7.4
Agent conservateur
Sodium azide
Précaution d'utilisation
This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Stock
-20 °C
Stockage commentaire
Store at -20°C. Avoid freeze / thaw cycles.
Antigène
TIMM8A/DDP (TIMM8A)
(Translocase of Inner Mitochondrial Membrane 8A (TIMM8A))
Autre désignation
TIMM8A
Sujet
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.