TIMM8A/DDP Antikörper (AA 14-97)

Produktnummer ABIN7600039

Dieses Anti-TIMM8A/DDP-Antikörper ist ein Kaninchen Polyklonal-Antikörper zur Detektion von TIMM8A/DDP in WB, ELISA, IHC, IF und ICC. Geeignet für Human, Maus, Ratte und Affe.

Kurzübersicht für TIMM8A/DDP Antikörper (AA 14-97) (ABIN7600039)

Target: TIMM8A/DDP (TIMM8A) (Translocase of Inner Mitochondrial Membrane 8A (TIMM8A))

Reaktivität: Human, Maus, Ratte, Affe

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser TIMM8A/DDP Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA, Immunohistochemistry (IHC), Immunofluorescence (IF), Immunocytochemistry (ICC)

Produktdetails anti-TIMM8A/DDP Antikörper

Bindungsspezifität: AA 14-97

Verwendungszweck: Anti-TIMM8A/DDP Antibody Picoband®

Kreuzreaktivität (Details): No cross-reactivity with other proteins.

Produktmerkmale: Anti-TIMM8A/DDP Antibody Picoband® (ABIN7600039). Tested in ELISA, IF, IHC, ICC, WB applications. This antibody reacts with Human, Monkey, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Aufreinigung: Immunogen affinity purified.

Immunogen: E.coli-derived human TIMM8A/DDP recombinant protein (Position: A14-D97).

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: Western blot, 0.25-0.5 μg/mL, Human, Mouse, Rat, Monkey
Immunohistochemistry (Paraffin-embedded Section), 2-5 μg/mL, Human, Rat
Immunocytochemistry/Immunofluorescence, 5 μg/mL, Human
ELISA, 0.1-0.5 μg/mL, -
1. Aguirre, L. A., del Castillo, I., Macaya, A., Meda, C., Villamar, M., Moreno-Pelayo, M. A., Moreno, F. A novel mutation in the gene encoding TIMM8a, a component of the mitochondrial protein translocase complexes, in a Spanish familial case of deafness-dystonia (Mohr-Tranebjaerg) syndrome. Am. J. Med. Genet. 140A: 392-397, 2006. 2. Ezquerra, M., Campdelacreu, J., Munoz, E., Tolosa, E., Marti, M. J. A novel intronic mutation in the DDP1 gene in a family with X-linked dystonia-deafness syndrome. Arch. Neurol. 62: 306-308, 2005. 3. Hofmann, S., Rothbauer, U., Muhlenbein, N., Neupert, W., Gerbitz, K.-D., Brunner, M., Bauer, M. F. The C66W mutation in the deafness dystonia peptide 1 (DDP1) affects the formation of functional DDP1-TIM13 complexes in the mitochondrial intermembrane space. J. Biol. Chem. 277: 23287-23293, 2002.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Konzentration: 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.01 mg Sodium azide.

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.

Details zu TIMM8A/DDP

Target: TIMM8A/DDP (TIMM8A) (Translocase of Inner Mitochondrial Membrane 8A (TIMM8A))

Andere Bezeichnung: TIMM8A

Hintergrund:

Synonyms: Cadherin-4, Retinal cadherin, R-CAD, R-cadherin, CDH4

Tissue Specificity: Expressed mainly in brain but also found in other tissues.

Background: Mitochondrial import inner membrane translocase subunit Tim8 A, also known as Deafness-dystonia peptide or protein is an enzyme that in humans is encoded by the TIMM8A gene. 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.

Molekulargewicht: 13-14 kDa

Gen-ID: 1678

UniProt: O60220