TIMM8A Polyclonal Antibody
规格: | / 20μL / 60μL / 120μL / 200μL |
价格: | / ¥420 / ¥1010 / ¥1640 / ¥2365 |
货号:E-AB-18755
宿主: Rabbit
反应性: H,M,R
应用: IHC
Overview
Synonyms | DDP 1,DDP,DDP1,Deafness dystonia protein 1,Deafness/dystonia peptide,DFN 1,DFN1,MGC12262,Mitochondrial import inner membrane translocase subunit Tim8 A,MTS,TIM 8A,TIM8,TIM8A,TIM8A,TIMM 8A,timm8a,Translocase of inner mitochondrial membrane 8 homolog A,X linked deafness dystonia protein,X-linked deafness dystonia protein,TIMM8A抗体 |
Swissprot | O60220 |
Source | Rabbit |
Reactivity | Human,Mouse,Rat |
Immunogen | Fusion protein of human TIMM8A |
Application | IHC(Detection kit: E-IR-R213) |
Recommended dilution | IHC,,1:40-1:200 |
Concentration | 0.84mg/mL |
Clonality | Polyclonal |
Properties
Cellular localization | Mitochondrion inner membrane. |
Isotype | IgG |
Purification | Affinity purification |
Conjugation | Unconjugated |
Storage instructions | Store at -20℃. Avoid freeze / thaw cycles. |
Storage buffer | PBS with 0.05% NaN3 and 40% Glycerol,pH7.4 |
Background | 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. |